Pathology

Question 1

T/F: all ApoB containing lipoproteins are atherogenic

Answer 1

It’s called ApoB 100 unless it’s in the chylomicron, in which case it’s ApoB48
mn: Apo -B - for Bad

Question 2

The most common cardiovascular abnormalities in Turner syndrome are

Answer 2

bicuspid aortic valve (a/w sudden cardiac death), coarctation of the aorta, and aortic dissection.

Question 3

The most common cardiovascular structures involved in Marfan syndrome are

Answer 3

the aorta and valves of the left heart (e.g., aortic root dilation, aortic regurgitation, mitral valve prolapse).

Question 4

The granulomatous vasculitis are:

Answer 4

1. ALL of the large vessel vasculitis’
   - Giant cell arteritis
   - Takayasu
2. NONE of the medium vessel vasculitis’
3. TWO of the four small vessel vasculitis
   - Wegener
   - Churg-strauss

Question 5

pathophys of achondroplasia

Answer 5

Mutations that result in constitutive activation of FGFR3 cause achondroplasia, a condition characterized by deficient longitudinal bone growth that manifests with short limbs and stature.

Question 6

decreased iodine uptake is associated with HYPER or HYPO - thyroidism?

Answer 6

in some forms of HYPER-thyroidism –> because there’s already enough thyroid hormone being produced (in order to cause the symptoms), so there’s negative feedback causing decreased requirements for iodine/making new thyroid hormone

exception = Grave’s disease, because thyroid hormone production is upregulated at the level of the TSH receptor

Question 7

what is Eisenmenger syndrome

Answer 7

Eisenmenger syndrome; can occur at any age

1. Prolonged pulmonary hypertension due to a left-to-right shunt causes reactive constriction with permanent remodeling of pulmonary vessels → irreversible pulmonary hypertension
2. Right ventricle hypertrophies to compensate for pulmonary hypertension → right ventricular pressure increasing and eventually exceeding left ventricular pressure → reversal of blood flow → onset of cyanosis; digital clubbing, and polycythemia

Question 8

common causes of orthostatic hypotension

Answer 8

Medications (alpha1 adrenergic antagonists, diuretics), volume depletion (d/t hemorrhage, hyperglycemia, or diuretics), autonomic dysfunction (e.g. parkinsons and diabetes)

Question 9

what is the feared outcome of low Apgar scores?

Answer 9

cerebral palsy

Question 10

what is Stress hyperglycemia

Answer 10

Stress hyperglycemia is transiently elevated blood glucose levels in the context of severe illness (eg, sepsis, burns, major hemorrhage) in patients without preexisting diabetes mellitus. Cortisol, catecholamines, and glucagon released in response to severe metabolic stress act on the liver to increase glycogenolysis and gluconeogenesis.

Question 11

Turner syndrome. cardiovascular abnormalities

Answer 11

The 45,XO genotype causes Turner syndrome. The most common cardiovascular abnormalities in Turner syndrome are bicuspid aortic valve, coarctation of the aorta, and aortic dissection.

Question 12

mitral stenosis most common etiology

Answer 12

rheumatic heart disease

Question 13

T/F: hyperparathyroidism can cause osteoporosis

Answer 13

yes, because T4 stimulates osteoclast activity

Question 14

signs and symptoms of aortic coarctation

Answer 14

• claudication with exertion (d/t decreased lower extremity blood pressure
• delayed femoral pulses
• high pressure in the upper extremity and head/neck

XR sign: notched ribs

Question 15

acute hypocalcemia signs and symptoms

Answer 15

tetany (Trousseau sign)
paresthesias (perioral tingling)
abdominal discomfort
diarrhea
QT prolongation

Question 16

hypocalcemia differential

Answer 16

• iatrogenic hypoparathyroidism (caused by trauma during thyroidectomy or parathyroidectomy)
• vitamin D deficiency
• PTH resistance
• autoimmune hypoparathyroidism
• increased phosphate binding (rhabdomyolysis or tumor lysis syndrome)
• hypomagnesemia.

Question 17

cardiac tamponade triad

Answer 17

muffled heart sounds, JVD, systemic hypotension

mn: go to MJ’S House to get a tampon

Question 18

mitral stenosis commonly occurs as a late complication of _________________

Answer 18

MS commonly occurs as a late complication of rheumatic fever, which is more common in developing countries

Question 19

epidemiology of myxoma

Answer 19

mn: adult hearts are LEFT with Myxed feelings

most common ages 40-60 in males,
usually left atrium
most common primary cardiac tumor
sporadic etiology

Question 20

hamartomas commonly occur with what genetic condition?

Answer 20

tuberous sclerosis (d/t mutations in TSC1 or TSC2 tumor suppressor genes)

ps. hamartoma is just a regular tissue that developed wrong rather than a true neoplasm

Question 21

_______________ is the radiologic modality of choice for evaluating pericardial effusions

Answer 21

3D Echocardiography

Prior to performing this, typically you’d have a chest XR with water bottle sign (enlarged heart with specific shape)

Question 23

Beck Triad

Answer 23

Beck triad of cardiac tamponade (“becky has a tampon”)

1. Hypotension
2. Muffled heart sounds
3. Distended neck veins

Question 24

Meckel’s diverticulum

Answer 24

Meckel’s Diverticulum = outpouching of gut at ilium d/t vitalline duct remnant

• Considered a true diverticulum (contains all layers of bowel incl muscular layer)
• Can contain ectopic gastric tissue
• Sx of melena, periumbilical pain, ulcerb (esp if gastric acid secreted) but often discovered incidentally
• Dx via Technetium scan (taken up by parietal cells of gastric tissue)

Rule of 2s: (mn)

1. 2% if population
2. M:F = 2:1
3. Within 2 feet of ileocecal valve
4. ~2” in size
5. Two –> “true” diverticulum
6. two types of tissue
7. “T” for two and technetium scan

Question 25

what’s this finding?

Answer 25

double bubble sign a/w duodenal atresia

n.b Bilious vomitting develops after the first feed as there is no lumen for flow of intestinal contents.

Question 26

duodenal atresia vs malrotation with volvulus

Answer 26

1. duodenal atresia:
   
   • bilious vomiting after 1st feed
   • XR shows double bubble sign
   • a.w down syndrome
2. In malrotation, the cecum is positioned in the right mid- to upper quadrant instead of the right lower quadrant. At birth, this can be asymptomatic. As the baby feeds and peristalsis occurs, malrotation often leads to volvulus, a twisting of the intestines around the mesentery –> intestinal obstruction develops with symptoms of bilious emesis and abdominal pain. An abdominal x-ray is unreliable for diagnosis and is often normal. Upper GI contrast study with fluoroscopy is the test of choice. Treatment is surgical.

Question 27

Good pasture syndrome vs granulomatosis with polyangiitis (GPA) or Micorscopic polyangiitis

Answer 27

all three have lower resporatory tract sx and glomerulonephritis.

but only GPA has upper respiratory tract sx

and microscopic polyangiitis is precepitated by medications

Question 28

pathogenesis of hepatic encephalopathy

Answer 28

↓ hepatic metabolism → accumulation of ammonia (NH3) → increased conversion of glutamate to glutamine (b/c ammonia is used for this conversion and therefore pushes this reaction forward)-> accumulation of glutamine in astrocytes (NOT neurons), causing swelling and dysfunctional release –> less glutamine delivered to neurons –> disruption of excitatory neurostransmition + cerebral edema and ↑ intracranial pressure → neurological deterioration

Question 29

what portocaval anastomosis site has the most dangerous associated pathology

Answer 29

esophageal varices because if they rupture and bleed it can be fatal

vs caput medusae or hemarrhoids

Question 30

pathogenesis of achalasia

Answer 30

decreased inhibitory neurons in the myenteric plexus (NOT the submucosal plexus) at the LES

Question 31

what is SMA syndrome

Answer 31

Superior mesenteric artery (SMA) syndrome is characterized by compression of the third portion of the duodenum between the aorta and the superior mesenteric artery. This results in intermittent duodenal obstruction. This often develops after significant weight loss which causes loss of mesenteric fat. Clinical manifestations of SMA syndrome include postprandial epigastric pain and early satiety, or more rarely bowel obstruction.

Question 32

if goblet cells are found in the lining of the stomach, what does that mean?

Answer 32

they are not normally there, they start at the duodenum, so if they are present, that means the stomach wall is undergoing metaplasia

Question 33

failure to pass meconium key ddx:

Answer 33

Hirschsprung’s disease and cystic fibrosis.

less likely are imperforate anus and failed recanaliztion of small bowel

n/b: in CF, you get Meconium ileus which is bowel obstruction due to thick, dry (inspissated) meconium.

(also imperforate anus, but this is less common therefore less likely)

Question 34

duodenal ulcers vs gastric ulcers

Answer 34

1. pain of duodenal ulcers decreases after a meal. By contrast, the pain of gastric ulcers usually increases with meals
2. Duodenal ulcers are almost always associated with hypersecretion of gastric acid. Gastric ulcers, by contrast, may or may not be associated with such hypersecretion. Thus hyperplasia of the Brunner glands is expected in a patient with a duodenal ulcer, as these glands help neutralize stomach acid.n/b for both types of ulcers
   
   1. there will probably be a hx of NSAID use (or look for pt with osteoarthritis, where nsaid use is implied)

Question 35

in barret’s esophagus, the tissue transforms into what tissue type?

Answer 35

into duodenal tissue, therefore you’d see Brunner’s glands which are unique to duodenum

Question 36

common features of small bowel obstruction

Answer 36

nausea, bilious vomiting, abdominal distention, and succussion splash (a sloshing sound heard on abdominal auscultation after moving the patient suddenly).

Question 37

drug induced esophagitis causes

Answer 37

NSAIDs, bisphosphonates, tetracyclines, potassium chloride, and iron,

Question 38

mallory-weiss vs Boerhaave syn.

Answer 38

Mallory weiss - not full thickness, present in “weekend warrior” (isolated alcoholic binge) +hematemesis

Boerhaave - full thickness tear, pneumomediastinum, chronic alcoholism

Question 39

skin manifestations in endocarditis (of any cause)

Answer 39

osler nodes, janeway lesions, roth spots, splinter hemorrhages

1. Osler nodes: painful red/violaceous nodules usually found on the pads of fingers and toes;
2. Janeway lesions: non painful red macules found on the palms and soles;
3. Roth spots: a red hemorrhagic lesion of the retina with a characteristic white center representing fibrin-platelet plugs;
4. Splinter hemorrhages: subungual hemorrhages appearing as linear non blanching brown-red lesions on the nail beds.

Question 40

describe the typical aortic regurgitation murmur

Answer 40

decrescendo early-diastolic blowing murmur, best heard on the left lower sternal border, around the 3rd and 4th intercostal spaces

Question 41

S3 gallops are normal in what types of patients

Answer 41

pregnant women and athletes (both of whom have high-output states physiologically) and children.

Question 42

Mutation in KRAS is a/w

Answer 42

activating mutation in the oncogene KRAS causes pancreatic ductal adenocarcinoma

Question 43

Mutation in BRAF is a/w

Answer 43

malignant melanoma

mn: BRAF –> Bratt –> meanie (starts with M)

Question 44

Mutation in BCL2 is a/w

Answer 44

follicular lymphoma and DLBL (diffuse large B cell lymphoma)

Bcl-2 normally inhibits apoptosis. when it gets overexpressed, you get insufficient apoptosis and uncontrolled cell growth.

Occurs via t(14;18) translocation

mn: I’m Falling (Foll) BeCause Love (BCL)

Question 45

Mutation in c-MYC is a/w

Answer 45

Burkitt lymphoma –> facial mass with ascites

d/t overexpression of it; it’s a transcription factor that increases expression of cyclins and cyclin dependent kinases

Question 46

Mutation in APC is a/w

Answer 46

familial adenomatous polyposis

Question 47

pt with angina, reduced femoral pulses, and carotid bruit

what’s the dx?

Answer 47

diffuse atherosclerosis

Question 48

what are the possible causes of rapidly progressive GN (RPGN)

Answer 48

RPGN is a syndrome that is under the broader category of nephritic syndrome. Crescent formation (composed of proliferating parietal cells, lymphocytes, macrophages, and fibrin—on light microscopy is diagnostic regarless of cause)

causes:

1. the small vessel vasculitides (except Henoch-Schonlein Pupura, aka IgA vasculitis) -all pauci-immune (no IF pattern)
   
   • granulomatosis with polyangiits (GPA; mn: C is a bad grade for a GPA) (formerly Wegners). a/w C-Anca
   • microscopic polyangiitis a/w P-Anca
   • eosinophilic granulomatosis with polyangiitis (formerly Churg Straus) a/w P-Anca (+asthma)
2. Goodpasture syndrome (ch/b linear IgG deposits + anti Collagen type 4 ab + complement along the GBM and alveaolor basement membrane). a/w hematuria + hemoptysis. No ANCA or ANA assoc.
3. the “S” related causes: both cause diffuse proliferative glomerulonephritis d/t random deposition of antigen-ab complexes –> granular pattern on IF
   
   1. strep - PSGN
   2. SLE - lupus nephritis –> ant-nuclear ab (ANA)

Question 49

“diffuse capillary wall thickening” is A/W what nephrotic syndrome

Answer 49

membranous nephropathy

*don’t confuse with “diffuse GBM thickening” as happens in Alport syndrome (alternating thinning and thickening)

Question 50

pathogenesis of gallstones in Crohn’s disease

Answer 50

mucosa of terminal ileum inflamed –> dysfunctional bile acid reabsorption –> bile acids lost in feces –> not enough bile acids present to emulsify fat so the cholesterol/bile acid ratio increases –> supersaturation of bile with cholesterol causes gallstones

Question 51

​​​​​​​Abnormal separation of the dorsal (gastrointestinal tract) and ventral (respiratory tract) foregut results in __________________________________________

Symptoms include respiratory distress and choking with feeds; chest x-ray would not show cystic thoracic structures.

Answer 51

esophageal atresia with or without a tracheoesophageal fistula.

Question 52

failure of closure of the pleuroperitoneal fold results in _______

Answer 52

congenital diaphragmatic hernia

sx: newborn with respiratory distress and decreased breath sounds unilaterally from pulmonary hypoplasia +scaphoid abdomen with barrel chest +chest XR with cystic features

Question 53

clinical features of Kallmann syndrome (GnRH deficiency)

Answer 53

delayed puberty, small penis, anosmia, hearing loss, cleft palate

Question 54

situs inversus (reversal of side of organs) is associated with what autosomal recessive condition

Answer 54

Kartagener syndrome a form of primary ciliary dyskinesia

pt will also have hx of recurrent respiratory infections and infertility

Question 55

in what conditions can you see pulsus paradoxus

Answer 55

asthma (severe), COPD, constrictive pericarditis

Question 56

forceful expulsion of gas and/or stool on digital rectal exam of newborn baby with bilious emesis is highly specific for what disorder

Answer 56

Hirschsprung

Question 57

what is budd chiari syndrome

Answer 57

occlusion of hepatic vein (drains blood from liver/portal circulation into systemic circulation). liver biopsy shows centrilobular congestion and fibrosis

n.b. restrictive pericaraditis would present similarly (d/t blood backed up in systemic circulation)

Question 58

pt with portal htn but normal liver biopsy most likely has what dx as the underlying cause?

Answer 58

portal vein thrombosis (upstream of liver)

Question 59

what are the classic findings in a neonate born with congenital rubella syndrome

Answer 59

deafness, cataracts, blueberry muffin rash, patent ductus arteriosus

*PDA presents with machine like murmur

mn: it’s rude (rudella) to tak a machine gun on a duck (duct) hunt. it will make people deaf

Question 60

Which DSD (in the fetus) will cause the mother to undergo virilization?

Answer 60

aromatase deficiency

Question 61

what is the classic presentation for a pt with splenic infarction

Answer 61

Splenic infarction presents with left upper quadrant abdominal pain, left shoulder pain, and vomiting in a patient with a history of embolic disorders or hypercoagulability.

Question 62

What are all the congenital defects a/w turner syndrome (45, XO)

Answer 62

1. Horseshoe kidney
2. Coarctation of the aorta (mn: C is horseshoe shape) +/-aortic dissection
3. bicuspid aortic valve
4. streak gonads,
5. webbed neck,
6. cubitus valgus (increased carrying angle),
7. lowset hairline and ears

Question 63

SIADH present in patients with what proad categories of conditions

Answer 63

1. chronic physiologic stress (e.g. infection),
2. tumors (e.g. small cell carcinoma of lung),
3. medication adverse effect (e.g. carbamazepine)

Question 64

What do you see in the XR and what is the probable dx?

Answer 64

hyperinflated lungs with increased bronchial markings = c/w COPD

Question 65

what cardiac pathology is c/w with this change (orange) in pressure volume loop?

Answer 65

mitral regurg

Question 66

what cardiac pathology is c/w with this change (red) in pressure volume loop?

Answer 66

aortic regurg

Question 67

what cardiac pathology is c/w with this change (green) in pressure volume loop?

Answer 67

mitral stenosis

Question 68

what cardiac pathology is c/w with this change (orange) in pressure volume loop?

Answer 68

increased contractility

Question 69

“Equalization of cardiac chamber pressures” happens in what specific dx?

Answer 69

cardiac tamponade

Question 70

What are the extrapyramidal symptoms and when do they occur most commonly?

Answer 70

mn: ADAPT (the 1st generation psychotics sucked b/c of these sx so they had to ADAPT and make new ones)

1. Acute Dystonia (muscle contractions)
2. Akathisia (restlessness, pacing)
3. Parkinsonism (bradykinesia, rigidity, masked facies)
4. Tardive diskinesia (weird mouth movements)

these occur in administration of 1st gen antipsychotics:

haloperidol, pimozide, trifluoperazine, fluphenazine, thioridazine, chlorpromazine.

Question 71

causes of Dilated cardiomyopathy

Answer 71

mn: ABCCCD

hronic Alcohol overuse, Beriberi, Coxsackie B myocarditis, chronic Cocaine use, Chagas disease, and Doxorubicin toxicity.

Question 72

pt with orthopnea, rales, an S3 sound, edema, and an elevated type B natriuretic peptide. + lack of left ventricular hypertrophy. What’s the dx?

Answer 72

dilated cardiomyopathy

Question 73

Disarrayed myofibrils are pathognomonic for what condition

Answer 73

hypertrophic cardiomyopathy.

Question 74

What is an accelerated junctional rhythm (AJR) and when does it occur?

Answer 74

Accelerated junctional rhythm (AJR) occurs when the atrioventricular (AV) junctional pacemaker is depolarizing at a rate that exceeds the rate of the sinus node. It can happen in digoxin toxicity (classic), myocarditis, and in the setting of high-dose beta agonists0

Question 75

what is the most common cause of small bowel obstructions

Answer 75

surgical adhesions

(appears on XR as multiple dilated small bowel loops)

Question 76

infant or child with harsh, holosystolic murmur most prominent at the left lower sternal border without accompanying symptoms (e.g. cyanosis) that was not heard at birth

what’s the defect? what’s the differential?

Answer 76

VSD

(d/t incomplete closure/development of interventricular septum)

tricuspid regurg (rare in a young person)

Question 77

mitochondrial myopathies inheritance pattern

Answer 77

they are d/t mutation in mitochondrial DNA, so theyare inherited via maternal inheritance (both males and females can get the dz but only females can transmit it). Also, it exhibits hteroplasmy (mutation only affects some mitochondria, not others), so there are variable clinical presentations.

e.g. MELAS syndrome

Question 78

MELAS syndrome

Answer 78

MELAS is an inherited mitochondrial myopathy d/y mutations encoding for electron transport chain proteins (so, not enough ATP) ch/b the sx outlined by the name:

1. M: mitochondrial
2. E: encephalomyopathy
3. L: Lactic-
4. A: -Acidosis
5. S: stroke-like episodes (e.g. hemiparesis of the face, hemianopia, altered consciousness)

presents during childhood with fatigue and exercise intolerance +lactic acidosis. Labs: high CK and lactate. Muscle biopsy with ragged red fibers

Question 79

cause of myotonic dystrophies

Answer 79

autosomal dominant mutations (exhibiting anticipation) affecting:

1. DMPK gene (type 1, congenital or adult) causing repeat expansion of CTG sequences
2. CNBP gene (type 2, adult form only) causing repeat expansion of CCTG sequences

^both cases causemore hydrogen bonding between C and G –> RNA forms into clumps –> attract RNA binding proteins, so some genes not accessible for transcription –> more protein produced

Question 80

This patient with myoclonic epilepsy, a positive maternal family history of myopathies, and muscle biopsy findings demonstrating ragged muscle fibers has a presentation consistent with what disease

Answer 80

mitochondrial myopathy

Question 81

myasthenia gravis is caused by __________

Answer 81

antibodies against the

acetylcholine receptors

on the postsynaptic membrane.

sx: fluctuating weakness and fatigability of ocular muscles

Question 82

diabetes insipidus vs SIADH

Answer 82

Both are due to hypothalamus secretions

diabetes insipidus –> decreased vasopressin/ADH –> water not reabsorbed, but salt doesn’t go with it –> hyperosmotic volume contraction

SIADH –> excess ADH –> water is resorbed but salt conc doesn’t change –> theoretical hyposomotic volume expansion BUT compensatory mechanisms kick in (aldosterone and natriueretic peptides) –> euvolemia

Question 83

sparing of the muscles of the forehead in facial paralysis indicates what

Answer 83

corticobulbar tract is affected instead of the facial nucleus or nerve

Question 84

why is a person with PCOS at risk for endometrial hyperplasia

Answer 84

It happens by 3 parallel processes that all contribute to the same problem:

1. Increased androgen production (male hormone) –> triggers HP axis to upregulate LH –> LH/FSH ratio elevated –> ovulation inhibited
2. Obesity, which is common with PCOS –> induces hyperinsulinism,–> acts synergistically in the ovary with LH to upregulate androgen production.
3. ​​​​​​​PCOS causes increased estrogen levels due to androgen-to-estrogen conversion by the enzyme aromatase within the ovary (eg, estradiol) and adipose tissue (eg, estrone). Estrogen normally stimulates endometrial proliferation to allow for implantation.

Question 85

t/f you see chronic pelvic in patients with PCOS

Answer 85

false

Question 86

what is the classic presentation of hyperplasa-based endometrial carcinoma?

Answer 86

women aged 55–65 with risk factors for unopposed estrogen with post-menopausal bleeding

workup with transurethral ultrasound, looking for increased in endometrial stripe, then do D&C if US is definitive. If suspicious for cancer, MRI and CT to stage it. on biopsy (which can involve D&C and therefore be therapeutic as well as diagnostic) you’d see increased gland to stroma ratio. Endometrial carcinoma does not metastasize, only grows. a/w PTEN mutation

vs serous pathway –> classic pt is an older black woman, +psamomma bodies a/w P53 mutation. worse prognosis

Question 87

Bulimia nervosa is characterized by recurrent binge eating/purging and preoccupation with body image, much like in many patients with AN. However, patients with bulimia maintain their body weight at or above the minimally normal level and do not experience the signs of starvation that are present in this patient.v

Question 88

presentation of ​​​​​​​Parvovirus categorized by age

Answer 88

fetus -hydrops fetalis

kids - “fifth disease” (erythema infectionsum) –> slapped cheek appearance

pts with sickle cell dz - aplastic crisis

adults - arthritis

Question 89

pt presents with HTN, given lisinopril (ACE inhibitor), then has signs of lower GFR (oliguria + elevated BUN and creatinine). what’s the dx?

Answer 89

renal artery stenosis

mechanism: because ACE inhibitors decrease ang II –> ang II preferentially constricts efferent arterioles to maintain GFR in spite of decreased renal artery perfusion in this case, so reduced ang II will cause a severe drop in GFR in these pts

Question 90

Lymphatic spread of infection is common in what types of infections

Answer 90

skin and soft tissue infections

Question 91

Mitral valve prolapse classically causes what heart sound change(s)

Answer 91

a midsystolic click followed by the late systolic murmur of mitral regurgitation (best heard at the cardiac apex). The click is relatively high pitched, and the timing varies with changes in LV blood volume.

Question 92

mononucleosis syndrome p/w:

Answer 92

fever, fatigue, sore throat, splenomegaly, atypical lymphocytosis

it is classically caused by Epstein barr virus (EBV) (dsDNA)

Question 93

What are the risk factors for gallbladder stasis

Answer 93

1. Pregnancy (d/t progesterone)
2. Rapid weight loss
3. prolonged parenteral nutrition
4. octreotide
5. upper spinal cord injuries

*n.b. gallbladder stasis is the pathogenesis of acalculous acute cholecystitis - classic prez is hospitalized pt.

Question 94

what pathogens can cause gallstones

(*this is not asking which ones get trapped in common bile duct)

Answer 94

ascaris lumbricoides: “knobby-coated, oval eggs” see pic

E. Coli: “Gram-negative, rod shaped, facultative anaerobe; virulence factors of fimbriae, pili, K capsule, LPS endotoxin”

pathogenesis: organisms produce enzymes that hydrolyze conjugated bilirubin into unconjugated bilirubin

Question 95

pt with fever, positive Boas sign, and positive murphy’s sign. What’s the dx?

Answer 95

acute cholecystitis

boas sign = raiation of RUQ pain to scapula

Question 96

Although GH has direct effects on target tissue (eg, chondrogenesis, myocyte protein synthesis), its growth-promoting effects are primarily mediated by insulin-like growth factor-1 (IGF-1), which is released from ____________

Answer 96

the liver following stimulation of hepatic GH receptors.

Although IGF-1 is also produced locally in the hypothalamus to regulate central nervous system activity, this source does not contribute significantly to bone and soft-tissue growth

Question 97

what is the presentation of Reye’s syndrome (aspirin poisoning in kids)

Answer 97

(liver failure and encephalopathy)

Question 98

what are the possible sx assoc. with Fanconi syndrome

Answer 98

fanconi syndrome = DNA repair defect

P/w: short stature, thumb or radial defects, cafe-au-le spots, increased incidence of malignancies, aplastic anemia

Question 99

what are the poss. sx assoc with diamond-blackfan anemia

Answer 99

diamond blackfan = abnormal synthesis of ribosomes

growth retardation, craniofacial abnormalities (hypertelorism & flat nasal bridge), thumb abnormalities, predisposition to blood cancers, anemic sx

Question 100

is transferrin high or low when total iron (including stored iron) is low

Answer 100

Transferrin = marker of total iron in the body including stores, upregulated in low iron states

Question 101

classic triad of Wernicke’s encephalopathy

Answer 101

• oculomotor disturbances (nystagmus)
• ataxia
• confusion

can be precipitated during glucose infusion of a chronic alcoholic, who is thiamine deficient. Because glucose pushes forward glycolysis and TCA, rapidly depleting thiamine, which is required for many enzymes in glucose metabolism

Question 104

What are the two non-MI causes of sudden cardiac death (non MI, so usually in young pts)

Answer 104

Answer: Bicuspid aortic valve (an anatomical variant a/w turner syndrome) and HOCM (hypertrophic septum)

Question 105

complications of aortic stenosis

Answer 105

Heart failure with preserved ejection fraction (diastolic dysfunction), atrial fibrillation, and pulmonary hypertension are common complications of AS.

Question 106

α1-antitrypsin deficiency is characterized by what lung finding

Answer 106

panacinar emphysema

Question 107

repeated spontaneous miscarriages and thromboembolic event are suggestive of what dx

Answer 107

antiphospholipid syndrome (APS) (a subtype/cause of thrombocytopenia)

Question 108

Leser-Trélat sign

Answer 108

Multiple, sudden-onset seborrheic keratoses

caused by Activation of epidermal growth factor receptors

a/w Solid cancers (especially gastrointestinal adenocarcinoma)

Question 109

what is Trousseau syndrome (aka thrombophlebitis migrans)

Answer 109

superficial thrombophlebitis that is recurrent and migratory (i.e., occurring in various locations, resolving, then appearing elsewhere). Referred to as Trousseau syndrome if related to underlying malignancy (most commonly pancreatic adenocarcinoma and bronchial carcinoma

Question 110

Erythema nodosum - what is this?

Answer 110

Erythema nodosum is an inflammatory disorder. In many cases, the cause is unknown. In some cases, it may be triggered by strep throat or other infections.

Symptoms include flat, firm, hot, red, and painful lumps that usually appear on the shins. Later, they may fade and look more like a bruise and then resolve on their own.

Question 112

Hilar lymphadenopathy is associated with what specific condition(s)

Answer 112

1. fungal infections,
2. mycobacterial infections (TB)
3. sarcoidosis.
4. hodgkin lymphoma

Question 113

Erysipelas what is it

Answer 113

an infection of the superficial dermis and dermal lymphatic vessels that is typically deeply erythematous and painful, with tense edema and distinct borders +/- peau de orange appearnace

The most common cause of erysipelas is S pyogenes

Question 114

What’s the most common cause of pharyngitis?

Answer 114

Viruses, especially adenovirus.

NOT strep, although that’s the most common bacterial cause

Question 115

risk factors/causes for gout

Answer 115

Question 116

Accumulation of abnormally ubiquitinated TAR DNA-binding protein-43 (TDP-43), which normally functions as a transcription inhibitor and DNA repair protein, is associated with what conditions

Answer 116

both amyotrophic lateral sclerosis and frontotemporal dementia

Question 117

how do you treat malignant hyperthermia

Answer 117

malignant hyperthermia caused by Mutations In Ryanodine Receptor(RYR1)cause Ca2+ release from sarcoplasmic reticulum.

therefore it’s treated by dantrolene, which blocks ryanodine receptors to prevent release of Ca2+ from the SER

Question 118

Polyarteritis nodosa

what is it, what is distinctive about it

Answer 118

1. small to medium vessel (e.g., renal, coronary, and mesenteric arteries) vasculitis in middle age men A/W Hep B positivity (30%), renal microaneurysms, and transmural (tunica intima and tunica media, mn: PANmural inflammation) fibrinoid necrotizing inflammation (+leukocyte infiltration). p/w neurological sx (foot drop); spares the lungs.
2. Patients typically present with systemic symptoms (eg, fatigue, fever, weight loss), renal failure, and abdominal pain/bloody stools due to ischemia of the involved organs
3. “should be considered in young adults presenting with stroke or myocardial infarction. The diagnosis may be confirmed with a biopsy of involved tissue. Tx is corticosteroids, cyclophosphamide”

NOT autoimmune

Question 119

granulomatosis with polyangiitis

Question 121

Granulomatosis with polyangiitis GPA classic presentation

Answer 121

nasopharyngeal ulceration, sinusitis, hemoptysis, hematuria

Question 122

head bobbing, “pistol-shot” femoral pulses points to what diagnosis

Answer 122

chronic aortic regurgitation

Question 123

Fixed-split S2 is most closely associated with what heart condition

why: The right atrium has lower pressure than the left atrium, so blood flows through the defect from the left atrium to the right. The extra blood in the right atrium is then pumped into the right ventricle. The right ventricle pumps the excess blood across the pulmonic valve, delaying closure and prolonging P2. Those with ASD have a fixed-split S2 regardless of their respiratory status because the right ventricle always pumps excess blood.

Answer 123

atrial septal defect.

Question 124

paradoxical splitting (split S2 during expiration and a single S2 during inspiration) is characteristic of what condition(s)

Answer 124

aortic stenosis and left bundle branch block

Question 125

wide split of S2 is characteristic of what condition(s)

Answer 125

pulmonary arterial hypertension, right bundle branch block, pulmonary stenosis

Question 126

S3 (if not physiologic) is caused by what conditions

Answer 126

HF with reduced EF

thyrotoxicosis (+ other high output states)

Mitral or aortic regurg

however, it can be normal in young people and during pregnancy

Question 127

what conditions can cause Reynaud’s phenomonenon

Answer 127

1. lupus
2. progressive systemic sclerosis
3. Buerger disease (Thromboangiitis obliterans) a/w smoking
4. other collagen vascular diseases.

Question 128

which lymphoma type has a painless waxing and waning lymphadenopathy

Answer 128

follicular (t(14;18)–> BCL2)

Question 129

what is sezary syndrome

Answer 129

Sezary syndrome is a subtype of cutaneous T-cell lymphoma with leukemic and skin malignant T-cell involvement that often presents with lymphadenopathy, erythroderma, and pruritus. peripheral smear and skin biopsies showing characteristic lymphocytes with “cerebriform” nuclei, an elevated CD4+ cell count, and clinical findings of erythroderma, generalized lymphadenopathy, and pruritus.

Question 130

The most common low-grade B-cell lymphoma is __________

Answer 130

follicular

while the most common low-grade T-cell lymphomas are cutaneous T-cell lymphomas, such as mycosis fungoides.

Question 131

what is Human T-cell lymphotropic virus

Answer 131

An RNA virus that belongs to the family of retroviridae. . Involved in the pathogenesis of adult T-cell lymphoma and HTLV-associated myelopathy. This virus is endemic to Japan and the Caribbean. a/w IV drug use

Question 132

what is mycosis fungoides

Answer 132

Mycosis fungoides is an indolent, CD4+ cutaneous T-cell lymphoma that presents on the skin. It is characterized by scaly, pruritic, well-demarcated skin plaques and patches. Named after shape of skin lesions, but it’s NOT of fungal origin.

ch/by “cerebriform nuclei” on histo

can progress to Sézary syndrome, a cutaneous T-cell lymphoma with leukemic dissemination of mutated T cells can be an advanced form of mycosis fungoides or arise de novo.

Question 133

Giant cells with bilobed nuclei describe what kind of pathognomonic histopath (tissue biopsy)

Answer 133

Reed-sternberg cells in Hodgkin Lyphoma (CD15/30 positive, lack CD20)

Question 134

Erythrocytes with basophilic nuclear remnants describes what kind of histopath finding and what is the assoc dz

Answer 134

Howell-Jolly bodies - seen on peripheral blood smears taken from patients with asplenia or functional splenic disorders such as sickle cell disease.

Question 135

“Myeloblasts with azurophilic granules”

describes what kind of histopath finding and what disease(s) is assoc.?

Answer 135

Auer rods a/w AML

Question 136

“Plasma cells with intracytoplasmic inclusions”

describes what peripheral blood cell finding and what dz is likely

Answer 136

Plasma cells with intracytoplasmic inclusions formed from accumulated immunoglobulins are typically seen in plasma cell dyscrasias such as multiple myeloma.

Question 137

which lymphoma(s) is associated with EBV infection

Answer 137

Burkitt, Hodgkins and diffuse large b cell

Question 138

Neoplastic lymphocytes that stain positive for tartrate-resistant acid phosphatase (TRAP) and CD11c in a bone marrow biopsy are characteristic findings of ____________

Answer 138

hairy cell leukemia.

Question 139

Hairy cell leukemia classic prez

Answer 139

It manifests with symptomatic anemia and thrombocytopenia, splenomegaly, and (rarely) B symptoms,

Question 140

AML and ALL classic prez

with

Answer 140

cytopenia, splenomegaly, and B symptoms are commonly seen,+ hepatomegaly and a rapid progression of the disease

Question 141

most common type of non indolent non-Hodgkin lymphoma in adults

Answer 141

diffuse large B cell

follicular lymphoma is the most common indolent type (presenting slowly)

Question 142

what cell types are increased in polycythemia vera

Answer 142

A chronic myeloproliferative neoplasm characterized by an irreversible increase in all myeloid lines: erythrocyte (predominates), granulocyte, and platelet

due to a mutation in the JAK2 gene. The increased blood cell mass leads to hyperviscosity, increasing the risk of thrombosis and poor oxygenation.

Question 143

t/f: only CML can progress to AML

Answer 143

false, all myeloproligerative disorders can progress to AML

(essential thrombocytopenia, polycythemia vera, CML, primary myelofibrosis)

Question 144

what mutation is involved in the myeloproliferative disorders

Answer 144

JAK2 mutations (Except fro CML, which has the BCR-ABL mutation)

Question 145

what conditions are a/w teardrop cells (dacrocytes)

Answer 145

myelofibrosis and thallassemia (b/c both have extramedullary hematopoesis)

Question 146

what is Erythroblastosis fetalis

Answer 146

an autoimmune hemolytic anemia that is most likely caused by Rh incompatibility and may present with jaundice, hepatosplenomegaly, and respiratory distress. When the mother had her first child, she was likely Rh negative and the child was Rh positive, resulting in the formation of maternal IgG antibodies that can affect future pregnancies.

Question 147

what dzs are a/w autoimmune regulator protein (AIRE) dysfunction?

“Life is ACHe without AIRE”

Answer 147

1. Adrenal insufficiency,
2. chronic mucocutaneous Candidiasis
3. Hypoparathyroidism

Question 148

describe Chronic granulomatous disease

Answer 148

a rare, XR, primary immunodeficiency of phagocytes caused by deficiency in NADPH oxidase in neutrophils and macrophages. –> Defective superoxide production –> susceptibility to cat. pos. infections

Question 149

what are the mutations associated with colon cancer

Answer 149

Colon cancer is associated with many gene mutations, including

APC, KRAS, RAF, and p53 mutations.

Question 150

baby with non bloody vomiting and diarrhea with the duodenal biopsy as shown. what’s the dx

Answer 150

Celiac because it’s ch/b vilous atrophy of the duodenum

Question 151

Erythema multiforme

what is it

what causes it

Answer 151

is a rash characterized by varying types of lesions, classically with target lesions (ring surrounding a dusky center).

causes:

1. infectious: Mycoplasma pneumoniae and HSV,
2. drugs: phenytoin, NSAIDs, beta-lactam antibiotics,

Question 152

myocardial amyloid deposition usually causes what kind of cardiomyopathy

Answer 152

restrictive cardiomyopathy,

Question 153

presentation of IgA vasculitis (also called Henoch-Schönlein purpura)

Answer 153

IgA vasculitis (also called Henoch-Schönlein purpura) presents with purpuric rash, abdominal pain, arthritis, and nephritic syndrome (IgA nephropathy/Berger dz)

often follows Upper respiratory infection (d/t mucus secretion –> increase IgA)

spares the lungs

Question 154

pt with spider angiomas, ataxia, and IgA def what’s the dx

Answer 154

Ataxia telangiectasia syndrome - a form of B cell immunodeficiency d/t defect in ATM gene causeing failure to detect DNA damage. AR inheritance

a/w increased AFP; high sensitivity to radiation

Question 155

alport syndrome

Answer 155

Hereditary nephritis (HN) (aka Alport syndrome), is defined as a spectrum of disease caused by mutations in genes encoding for type IV collagen (mn: al-FOURt) and resulting in thinning of the glomerular basement membrane commonly presenting in infants with gross hematuria as well as eye damage, and hearing loss.

It is X-linked in around 85% of cases, but it can also be autosomal recessive or autosomal dominant. Though rare, Alport syndrome is an important hereditary cause of end-stage renal disease (ESRD), as almost all men with the X-linked disease develop ESRD by middle age

One particular condition classically associated with Alport syndrome is anterior lenticonus, the outward bulging of the lens through the pupil.

Question 156

sx of maple syrup urine dz

Answer 156

dystonia, irritability, poor feeding, vomiting, lethargy, and seizures.

Question 157

what are degmacytes

Answer 157

bite cells as seen in G6PD deficiency (macrophages try to bite out the Heinz bodies)

mn: they “deg” them out

Question 158

Biliary obstruction alone typically leads to ___________ (direct or indirect) hyperbilirubinemia,

Answer 158

direct (conjugated)

as a mechanical blockage prevents normal excretion of conjugated bilirubin.

Question 159

Absence of multidrug resistance protein 2 is typical of what genetic disease

Answer 159

Dubin-Johnson syndrome. This protein transports bilirubin from the hepatocytes, after conjugation, into the bile collecting system. These patients can present with mild jaundice, but the hyperbilirubinemia is predominantly direct.

Question 160

____________is a disorder in which the only significant abnormality is mild, unconjugated hyperbilirubinemia that manifests as jaundice in times of stress, such as fasting or gastrointestinal illness.

Answer 160

Gilbert syndrome

in contrast to Crigler-Najjar syndrome, which is more severe

Question 161

If a pt has skin yellowing but no scleral icterus, what could be the dx

Answer 161

scleral icteris occurs before jaundice, therefore if it is not present, it’s a sign that the pt doesn’t have hyperbilirubinemia and they have pseudojaundice instead. Alternative cause would be elevated beta carotene levels (carotenemia) usually due to dietary intake (hypothyroidism causes reduced production of vit A thereby increasing beta carotene levels)

Question 162

strongest risk factor for pancreatic adenocarcinoma

Answer 162

smoking

Question 163

Pt with weight loss, smoking hx, abdominal pain, and obstructive jaundice with palpable non-tender gallbladder

what’s the dx

Answer 163

probably pancreatic adenocarcinoma of pancreas head blocking the common bile duct

why:

smoking is the #1 risk factor

weight loss is due to malabsorption (from decreased pancreatic enzymes)

a/w CA19-9 and CEA

Question 164

struvite stones (ammonium magnesium phosphate) are a/w which conditions

Answer 164

infection with urease pos. bugs:

proteus mirabilis, staph sapro, klebsiella

hydrolyze urea to ammonia –> alkalization of urine

a/w Staghorn calculi and sarcophagus shaped stones

Question 165

in what dzs would you see uric acid kidney stones

Answer 165

makes up 5% of stones overall, but would be most likely in pts with:

1. gout
2. dx with increased cell turnover (e.g. Leukemia) mn: l-U-kemia

radiol-U-cent on XR (unlike the other types of stones, except cystine stones which are also somewhat radiolucent); treated with allopurinol

Question 166

what is the most common type of kidney stones and what causes them

Answer 166

Calcium stones (oxalate or phosphate)

causes: antifreeze ingestion, vitamin C overuse, Crohn’s dz (malabsorption)

radiopaque on XR, tx with thiazides, citrate, low sodium diet.

Question 167

what is the prez of NF2

Answer 167

NF2 presents with benign neurologic tumors (vestibular schwannomas, meningiomas of the brain and spine), eye issues (cataracts), and cutaneous lesions (tumors, plaques).

Question 168

classic prez of neuroblastoma

Answer 168

Neuroblastomas affect children younger than 4 years more often than any other age group. The child may present with abdominal distention and a palpable, irregular abdominal mass that tends to cross the midline (Figure 1). Commonly, the child’s parent notices the abdominal mass while bathing the child. Neuroblastomas grow rapidly and may cause hypertension because of compression of the renal artery or secondary to catecholamines release. In addition, children with neuroblastoma can develop opsoclonus-myoclonus syndrome (dancing eyes, dancing feet). A concerned parent may explain that the child’s eyes have been moving fast and the child’s muscles have been twitching.

Question 169

2-year-old boy presents to the pediatrician after his mother noticed an irregular abdominal mass crossing the midline. On exam, the physician notes the patient’s dancing eye movements and unpredictable leg twitching.

what’s the dx?

Answer 169

neuroblastoma

Question 170

CD19, CD20, and CD5+ immunophenotype is characteristic of what lymphoproliferative disorder?

Answer 170

CLL

Question 171

Malignant vascular tumors such as hemangiosarcomas usually have a primary origin in the ____

Answer 171

spleen.

Question 172

Fanconi syndrome

what dzs can cause it

Answer 172

Hereditary (all cause toxicity to PCT, leading to the syndrome)

1. Wilson’s disease
2. Tyrosinemia
3. Glycogen storage disease

Acquired:

1. hypovolemia –>ischemia
2. multiple myoloma
3. cisplatin, lead

n.b. this is the only renal tubular defect that can be hereditary OR acquired (the others, Bartter, Gitelman, and Liddle are all AR inherited)

Question 173

what causes hereditary SAME

Answer 173

deficiency in 11-ß-hydroxysteroid dehydrogenase (degrades cortisol to cortisone, –> def. leads to excess cortisol accumulation)

as an acquired condition this can be caused by eating too much licorice

Question 174

__________should be suspected in patients with elevated creatinine, heme-positive urine, and no RBC seen on urinalysis.

Answer 174

Rhabdomyolysis-induced acute tubular necrosis

Rhabdomyolysis can be caused by trauma, hypo/hyperthermia, severe hypokalemia, or drugs like statins.

The classic triad of rhabdomyolysis includes myalgias, generalized weakness, and myoglobinuria (darkened urine) +/- muddy brown casts

Question 175

In a patient with systemic symptoms of fever, pruritis, wheezing, anorexia, and hepatomegaly, the diagnosis of ______________should be considered.

Answer 175

a nematode infection

Question 176

pt with hepatosplenomegaly, osteoporosis, pancytopenia, and bone pain crises +/- avascular necrosis.

You find out this is a lysosomal storage dz. Which one is it?

Answer 176

Gaucher disease is a lysosomal storage disease due to a deficiency in β-glucosidase (glucocerebrosidase) –> causes accumulation of Glucocerebroside

presence of lipid laden macrophages (as in Neimann Pick dx) but in this dz, they look like crumpled up “Gouged” tissue paper

Question 177

pt with coarse facial features, developmental regression, and hepatosplenomegaly.

you find out he has an X linked disorder caused by an enzyme deficiency. what is it?

Answer 177

Hunter syndrome is an X-linked recessive disorder caused by a deficiency of Iduronate-2-sulfatase.

if the pt had + corneal clouding, it would be Hurler syndrome, a deficiency in alpha - L -iduronidase

mn: Hunters see clearly (no corneal clouding) and aggressively aim for the X (X-linked recessive )

Question 178

pt with hepatosplenomegaly, foam cells, and red spots on the macula.

You find out they have a lysosomal storage dz, which one is it?

Answer 178

Niemann-Pick disease is a progressive neurodegenerative disease caused by a sphingomyelinase deficiency –> accumulation of sphingomyelin

*Tay-sachs also p/w cherry red macula spot, but NOT hepatomegaly

Question 179

pt with peripheral neuropathy, developmental regression, optic atrophy, and globoid cells.

you find out they have a lyosomal storage dz, which one is it

Answer 179

Krabbe disease is due to a deficiency in galactocerebrosidase; it can cause peripheral neuropathy, developmental regression, optic atrophy, and globoid cells. Causes accumulation of Galactocerebroside, psychosine

(mn: a crab is a constellation in the Galact-axy)

don’t confuse with Goucher, which is a def. in Glucocerebroside

Question 180

pt with developmental regression, hyperreflexia, hyperacusis, and a “cherry-red” macula spot with NO hepatomegaly

you find out they have a lysosomal storage dz. which one is it?

Answer 180

Tay-Sachs

caused by a deficiency in hexosaminidase-A causing accumulation of GM2 ganglioside

mn: HeXosaminidase A (“TAy-SaX”)

Question 181

histopathologic findings seen in dermatomyositis

Answer 181

• are infiltration of CD4+ T cells and complement proteins into the perimysium and small blood
• perifascicular atrophy and vasculitis

mn: ABCD –> 4th letter –> CD4+ t cells

Question 182

prez of fructokinase deficiency

Answer 182

a benign, asymptomatic disorder called essential fructosuria. The diagnosis may be confirmed when fructose is detected on urinalysis, but this deficiency does not typically manifest with any clinical symptoms.

Question 183

Deficiency of galactose-1-phosphate uridyltransferase prez.

Answer 183

Deficiency of galactose-1-phosphate uridyltransferase causes galactosemia, which manifests after ingestion of galactose in breast milk or formula

vs galactokinase deficiency, which presents later in life with only sx of cataracts

Question 184

aldolase B deficiency

Answer 184

hypoglycemia (esp after fruit juice) + symptoms of lethargy, tachypnea, hypothermia, and diaphoresis is most consistent with hereditary fructose intolerance.

Hereditary fructose intolerance is a disorder characterized by deficiency of aldolase B. An enzyme critical in converting fructose-1-phosphate to intermediary products that can be utilized in glycolysis. Accumulation of fructose-1-phosphate leads to a decrease in the available phosphate, which leads to inhibition of glycogenolysis and gluconeogenesis.

vs essential fructosuria: fructokinase deficiency (mn: is kinder)

Question 185

new born with low weight, jaundice, infantile cataracts, hepatomegaly, and vomiting after breastfeeding

what’s the dx

Answer 185

probably classic galactosemia caused by def of galactose-1-phosphate uridyltransferase

vs. A deficiency in galactokinase, which catalyzes the conversion of galactose to galactose-1-phosphate, causes infantile cataracts.

Question 186

deficiency in Aldose reductase is implicated in what chronic dz

Answer 186

end-organ damage in diabetes mellitus.

Question 187

which inborn error of metabolism is associated with increased risk of E. Coli infection

Answer 187

classic galactosemia caused by def of galactose-1-phosphate uridyltransferase

Question 188

Pulmonary hypoplasia happens in oligo- or polyhydramnios?

Answer 188

oligohydramnios - caused by the lack of normal alveolar distension by aspirated amniotic fluid.

part of Potter sequence

Question 189

what causes the sx in hereditary angioedema

Answer 189

an inherited disorder of deficient or dysfunctional C1 esterase inhibitor, leading to increased levels of bradykinin and C1 complement pathway products, which increase vascular dilation and permeability associated with angioedema.

n.b ACE breaks down bradykinin, and therefore ACE inhibitors should be avoided in patients with HAE due to increased swelling and risk of airway compromise.

Question 190

renal biopsy with nodular sclerosis (also called Kimmelstiel-Wilson nodules) is ch/of what type of nephropathy

Answer 190

advanced diabetic nephropathy

Early diabetic nephropathy is characterized by thickening of the glomerular basement membrane (GBM) and by diffuse mesangial cellular hyperplasia.

Question 191

Subepithelial immune deposits vs Subendothelial immune deposits vs Intramembranous immune deposits in Glomerular disease - wich dz causes which histopath

Answer 191

Subepithelial immune deposits can be seen in postinfectious GN or in membranous nephropathy

Subendothelial deposits are seen in lupus nephritis

mn Lupus is lower down

Intramembranous immune deposits are characteristic of membranoproliferative glomerulonephritis, which is mostly associated with hepatitis C infection

Question 192

The most common cause of systolic HF is

Answer 192

ischemic heart disease, which can occur with or without a prior myocardial infarction.

Question 193

presence of Q waves means

Answer 193

prior anterior myocardial infarction or dilated cardiomyopatphy

Question 194

A 56-year-old man with difficulty breathing for a few months dies suddenly on his way to the emergency department. Histologic findings in a lung tissue specimen obtained at autopsy are shown. dx?

Answer 194

Microscopic features of pulmonary edema as a result of heart failure include the presence of hemosiderin-laden macrophages.

Question 195

physical exam findings in pericardial effusion

Answer 195

Fluid in the pericardial space can cause cardiac tamponade, which presents with distant heart sounds, jugular venous distention, and hypotension; pulsus paradoxus (a decrease in systolic pressure by 10 mm Hg during inspiration) is often present.

Question 196

Constrictive pericarditis causes

Answer 196

Constrictive pericarditis is marked by the proliferation of fibrous tissue that causes a loss of elasticity in the pericardial tissues. It can lead to the signs of right-sided heart failure.

Causes include viral infection, post-radiation therapy, postsurgical scarring, systemic lupus erythematosus, and rheumatic fever

Question 197

what are Cutaneous neurofibromas and with what dz are they assoc.

Answer 197

usually manifest during early adolescence as multiple, raised, fleshy tumors (<2 cm) that often increase in size and number with age. These are benign nerve sheath neoplasms predominantly comprised of Schwann cells, which are embryologically derived from the neural crest.

neurofibromatosis type 1

Question 198

alpha-1 antitrypsin deficiency pathophys

Answer 198

Neutrophil-secreted elastase is an endogenous proteolytic enzyme that hydrolyzes elastin within alveolar walls. The liver synthesizes alpha-1 antitrypsin, a protein that inhibits neutrophil elastase and prevents alveolar wall degradation, particularly in the lower airways. Patients with alpha-1 antitrypsin deficiency consequently develop excessive alveolar elastin degradation, which clinically manifests with early-onset, lower lobe–predominant emphysema.

Question 199

newborn with hypoxemia and narrow mediastinal shadow

what’s the dx

Answer 199

transposition of the greater vessels

it’s a cyanotic heart defect that causes narrow medastinal shadow

also +/- murmur

Question 200

Third-degree (complete) AV block pathophys

Answer 200

Third-degree (complete) AV block involves a total lack of communication between the atria and ventricles due to AV node dysfunction. It can result from ischemia, infiltrative disease (eg, sarcoidosis), infection (eg, Lyme disease), or age-related fibrosis with cellular degeneration.

Question 201

auscultation findings in mitral stenosis

Answer 201

Classic physical examination findings include a loud first heart sound (S1), an early diastolic high-frequency opening snap after the second heart sound (S2), and a low-pitched diastolic rumble. These findings are best heard at the cardiac apex

The opening snap is caused by the sudden opening of the mitral valve leaflets when the left ventricular diastolic pressure falls below the left atrial pressure at the beginning of diastole.

Question 202

what valve is affected most commonly in IV drug assoc. infective endocarditis

Answer 202

Tricuspid

Injection drug use is associated with the concurrent injection of abrasive particulate matter and microorganisms into the bloodstream. Over time, injected particulate material denudes the surface of the heart valves, thereby creating a variegated surface for microorganism attachment. This dramatically increases the risk of infective endocarditis (IE). The right-sided valves are at greatest risk because pulmonary capillaries prevent large particulate matter from reaching the left side of the heart; the tricuspid valve is involved in >90% of cases.

usually the pathogen is staph aureus

don’t confuse with rheumatic fever, in which the mitral valve is most commonly affected

Question 203

histopath in TB

Answer 203

characterized by caseating granulomas (central necrosis is present) and acid-fast bacilli. Tuberculosis occurs more frequently in those with risk factors (eg, incarceration, homeless, high-risk health care workers)

Question 204

child tripodding with extended neck in setting of fever most likely indicates what dx?

Answer 204

epiglottitis

Question 205

newborn with chorioretinitis, hydrocephalus and diffuse intracranial calcifications

what’s the in utero infection?

Answer 205

toxoplasmosis

Question 206

newborn with rhinorrhea (sniphilis), skeletal anomalies, and deSquamating rash

what’s the in utero infection?

Answer 206

syphilis

Question 207

newborn with cataracts, heart defects (PDA), and sensorineural hearing loss.

what’s the in utero infection?

Answer 207

rubella

mn: RUDE-ella

Question 208

newborn with periventricular calcifications, microcephaly, and sensorineural hearing loss

what’s the in utero infection?

Answer 208

CMV

Question 209

newborn with vesicular/ulcerative rash

what’s the in utero infection

Answer 209

HSV

Question 210

what part of the brain is most susceptible to damage from global cerebral ischemia

Answer 210

Global cerebral ischemia is typically precipitated by systemic hypotension (eg, cardiac arrest). The cells most susceptible to ischemia are the CA1 pyramidal neurons of the hippocampus. Other areas of the brain that are damaged early in the course of global cerebral ischemia include the cerebellar Purkinje cells and the pyramidal neurons of the neocortex.

Question 211

What injuries affect the axillary nerve?

Answer 211

Anterior dislocation of the humerus and fracture of the surgical neck of the humerus are injuries affecting the axillary nerve.

Question 212

how to differentiate distal vs proximal radial nerve injuries

Answer 212

proximal: loss of wrist extension (wrist drop) often caused by mid shaft humerus fracture
distal: lateral forearm pain

Damage to the radial nerve can occur due to midshaft humeral fractures or compression of the axilla (eg, with the use of crutches).

Question 213

biceps weakness (decreased forearm flexion) what nerve root is damaged

Answer 213

C6

e.g. Erb’s palsy or C5-6 disc herniation

Question 214

finger abduction and adduction impaired

what nerve root is affected

Answer 214

T1

Question 215

finger flexion is impaired. what nerve root is affected

Answer 215

C8

Question 216

forearm extension (triceps) impaired and middle finger paresthesias. what nerve root is affected

Answer 216

C7 (e.g. d/t C6-7 herniation)

Question 217

upper arm abduction impaired + anesthesia over the shoulder

what nerve root is affeted

Answer 217

C5

impaires deltoid and teres minor muscles

Question 218

what nerve is most commonly damaged during a shoulder surgery

Answer 218

musculocutaneous nerve –> leading to weakness of upper arm flexion and impaired sensation over latereal forearm

Question 219

how to differentiate distal vs proximal median nerve injuries

Answer 219

proximal: loss of flexion of first three digits (pope’s blessing, when making fist). often caused by humerus supracondular fracture in kids)
distal: carpal tunnel syndrome or median claw
mn: M –> man –> makes a fist and only a man can be a pope

Question 220

Winging of the scapula

what nerves affected and how does this most commonly occur

Answer 220

long thoracic nerve (coming off of C5-7)

due to injury of the long thoracic nerve is most often caused by penetrating trauma to the upper lateral chest, such as with a stab wound, or from a mastectomy or axillary LN dissection

Question 221

The pain of acute appendicitis starts as visceral pain, which is carried by what nerve fibers

Answer 221

activated neurons of the T10 spinal cord segment.

Question 222

loss of the biceps tendon reflex, and sensory loss over the lateral forearm

what nerve is damaged

Answer 222

musculocutaneous

Question 223

hearing loss and multifocal bone pain with cortical thickening

what dx accounts for both of these findings

Answer 223

Paget dz of bone

high rate of bone remodeling: ↑ RANKL-RANK activity → ↑ NF-κB signaling → ↑ osteoclast activity → ↑ osteoblast activity → formation of disorganized (woven) bone

The pelvis, skull, vertebral column, and long bones of the lower extremities are the most commonly affected sites.

Skull involvement (in ∼ 40% of cases), leading to skull enlargement (increasing hat size), Cranial nerve deficits, Impaired hearing: due to ankylosis of the ossicles ; and narrowing of the internal auditory meatus

Question 224

____________-should be considered in an asymptomatic patient who presents with isolated ALP elevation that cannot be explained by any other means (e.g., cholestasis or bone metastases).

Answer 224

Paget disease

has Normal calcium, phosphate, and parathyroid hormone (PTH) levels

Question 225

tx for paget dx of bone

Answer 225

Bisphosphonates

inhibit osteoclast-mediated bone resorption. Used to treat hypercalcemia and bone metabolism disorders (e.g., osteoporosis or tumor-induced osteolysis).

Examples include zoledronic acid, pamidronate, etidronate, and alendronate.

Question 226

A characteristic skin biopsy finding in systemic sclerosis

Answer 226

dermal layer expansion due to diffuse deposition of collagen (eosinophilic connective tissue), resulting in atrophy of the intradermal adipose tissue and dermal appendages (ie, hair follicles, glands).

Question 227

CREST syndrome

Answer 227

name for the pentad of sx in limited scleroderma

Calcinosis cutis (pic)

anti-Centromere ab (mn)

Raynaud’s

Esophageal Dysmotility

Sclerodactyly

Telangiectasias

Question 228

what is thromboangiitis obliterans and how does it present

Answer 228

decreased distal pulses, dry gangrene, and ulceration over the digits. Together, these findings indicate thromboangiitis obliterans, also called Buerger disease. Thromboangiitis obliterans is an occluding inflammation of small to medium vessels and is not a true vasculitis because the vessel wall is not inflamed. Because the pathophysiology is essentially small- to medium-vessel ischemia, its clinical presentation mimics that of peripheral arterial disease, and distal pulses are decreased compared with proximal pulses.

smoking = biggest risk factor

Question 229

Bilateral lesions of the _____________cause Klüver-Bucy syndrome,

Answer 229

amygdala

characterized by hyperorality, hypersexuality, and behavioral disinhibition. This may be caused by herpes encephalitis.

Question 230

what part of the brain affected by Wernicke-Korsakov encephalopathy

Answer 230

mamillary bodies, d/t their high O2 requirements

Question 231

myasthenia gravis vs lambert eaton muasthenic syndrome - how do they differ in terms of clinical prez

Answer 231

Lambert-Eaton myasthenic syndrome manifests as proximal muscle weakness that improves with use + hyporeflexia. Myasthenia gravis presents as muscle weakness that worsens with use; reflexes spared.

Question 232

dermatomyositis vs polymyositis

how do they differ in terms of histopathology

Answer 232

The pathology of dermatomyositis is perimysial inflammation with perifascicular atrophy.

polymyositis = Endomysial infiltration of inflammatory infiltrate

mn: remember that perimysial lines the fascicles, just like skin lines the body

Question 233

what are the characteristic serum lab findings in Multiple myeloma

what are the characteristic urine findings

what does blood smear show

Answer 233

• blood: anemia, elevated calcium, +/- elevated creatinine (if there’s kidney damage, which often happens), ↑ β2 microglobulin
• urine electrophoresis: lambda light chains (aka Bence Jones Protein) (n.b. urine is foamy)
• Serum protein electrophoresis: (best initial test): monoclonal gammopathy with M protein (M spike) (NOT the same thing as IgM)
• blood smear: rollaux
• BMX: chromatin arranged in periphery of nucleus

MM should be suspected in elderly patients who have any combination of osteolytic lesions, hypercalcemia, anemia, or acute kidney injury (such as this patient). Other common symptoms include weight loss, constipation (due to hypercalcemia), fatigue, and recurrent infection

Renal disease is most commonly caused by light chain cast nephropathy, (excess free light chains exceed resorptive capacity of kidney and combine with Tamm Horsfall proteins to form eobstructive casts)

Question 234

what is the pathophys of annular pancreas

Answer 234

compression of the second part of the duodenum

may be asymptomatic or p/w biliary emesis

Question 235

the most common cancers (excluding basal and squamous cell skin cancer) in women by order of incidence are

Answer 235

breast, lung, and colorectal cancer.

Question 236

what lab values define renal insufficiency

Answer 236

creatinine clearance < 40 mL/min or serum creatinine > 2 mg/dL

Question 237

what triad characterizes Pellagra

Answer 237

dermatitis (in C3-C4 distribution), dementia, diarrhea

(mn: the 3 Ds of Vit B3 deficiency)

Pellagra can also be seen occasionally in those with carcinoid syndrome, prolonged isoniazid therapy, or Hartnup disease.

Question 238

what is the typical clinical prez of Hyper IgM syndrome

Answer 238

Hyper-IgM syndrome, in which immunoglobulin class-switching is impaired due to CD40 ligand deficiency, typically presents in infancy with sinopulmonary and opportunistic infections. Recurrent cold skin abscesses do not typically occur (vs Hyper IgE syndrome)

Question 239

Pyruvate kinase (PK) converts phosphoenolpyruvate to pyruvate during glycolysis. PK deficiency p/w _____________

Answer 239

causes chronic hemolytic anemia because glycolysis is the main source of energy for erythrocytes

Question 240

what is this heart sound

Answer 240

Split S2

Question 241

what spleen findings are a/w sickle cell dz

Answer 241

Repeated infarctions produce a shrunken, fibrotic spleen. By adulthood, most patients with SCD have undergone autosplenectomy and have a small, scarred splenic remnant. As a result, these patients have functional asplenia and are susceptible to infection with encapsulated organisms (eg, Streptococcus pneumoniae).

Question 242

unilateral itchy rash on nipple and aerola unrelieved by corticosteroids what’s the most likely dx?

Answer 242

This patient likely has Paget disease of the breast (PDB), a malignant breast condition characterized by a unilateral, painful/pruritic, eczematous rash confined to the nipple and areola. Associated scaling and ulceration are also frequently seen.

PDB is diagnosed by tissue biopsy (eg, wedge, punch) that reveals Paget cells in the epidermis of the nipple-areolar complex. Paget cells are malignant, intraepithelial adenocarcinoma cells that appear enlarged with abundant cytoplasm and prominent nucleoli on microscopy. These cells and associated skin changes are thought to be caused by the spread of an underlying breast malignancy (eg, intraductal carcinoma, ductal carcinoma in situ) to the nipple surface via the mammary ducts, resulting in the eczematous nipple rash.

Because approximately 85% of patients have an underlying breast malignancy, all patients with PDB require diagnostic mammography even without a palpable breast mass.

Question 243

what is a struma ovarii

Answer 243

Struma ovarii is an ovarian germ cell tumor composed of >50% mature thyroid tissue that can secrete thyroid hormone. Therefore, struma ovarii can be a rare cause of thyrotoxicosis (eg, weight loss, irregular menses, low TSH level).

On gross examination, struma ovarii typically appears as an oily (eg, sebaceous) cystic mass. Microscopic examination reveals thyroid follicles filled with colloid and surrounded by ovarian stroma.

Question 244

pediatric patient with atopy and frequent infections who recently had a skin infection and now presents with bloody urine and normal BUN/Creatinine.

What’s the dx?

Answer 244

post streptococcal glomerulonephritis

beacuse skin infections in someone with recurrent infections is more likely strep

Question 245

T/F: serum levels of calcium, phosphate and alk phos are normal in osteoporosis

Answer 245

TRUE

Question 246

_____________is indicated by an elevated alkaline phosphatase level in the setting of normal calcium and phosphate levels. This disease is characterized by increased osteoclast activity, followed by increased osteoblast activity.

Answer 246

Paget’s disease of bone

Elevated alkaline phosphatase is a sign of hyperactive osteoclastic bone resorption

Question 247

Hypercalcemia with normal phosphate and high alkaline phosphatase would be seen in ______________

Answer 247

metastatic cancer to the bones.

however *Note that malignancy usually causes hypercalcemia by secreting parathyroid hormone–like protein, which would act like hyperparathyroidism and cause hypercalcemia with hypophosphatemia.

vs hypercalcemia, decreased phosphate, and mildly elevated alkaline phosphatase levels seen in primary hyperparathyroidism

Question 248

pathogenesis of acute pancreatitis

Answer 248

Pancreatic zymogens are normally converted into their active form by trypsin in the duodenal lumen. Premature cleavage of trypsinogen to trypsin within the pancreas leads to uncontrolled activation of these zymogens, causing pancreatic autodigestion and acute pancreatitis.

Question 249

which cancers are most prone too metastasizing to the bone

Answer 249

breast, lung, thyroid, renal cell carcinoma, and prostate cancer.

Question 250

pathogenesis of ankylosing spondylitis

Answer 250

the inflammatory process is thought to originate in the gut due to an altered intestinal biome and defects in the mucosal barrier; the resulting inflammatory response leads to upregulation of cytokines (primarily tumor necrosis factor-alpha and IL-17), with activation of innate immunity and migration of immune cells to the skeleton. The resulting skeletal manifestations are most prominent in areas of mechanical stress, primarily at the insertions of tendons, ligaments, and joint capsules (collectively, entheses) on bone.

prez: back pain with onset <40 yo that is insidious with stiffness better with activity, worse with rest

Question 251

positive anti-tissue transglutaminase IgA antibodies indicates what disease

Answer 251

celiac dz –> villous atrophy –> decreased ADEK vitamin absorption

Question 252

what’s the most likely dx?

Answer 252

these features are characteristic of megaloblastic anemia

Question 253

what antibody is present in primary biliary cholangitis

Answer 253

anti-mitochondria

Question 254

causes of pill esophagitis

Answer 254

Pill esophagitis occurs when caustic medications come in contact with the esophageal mucosa for a prolonged time, leading to direct mucosal injury. Common culprits include tetracyclines (eg, doxycycline), bisphosphonates (eg, alendronate), potassium chloride, and nonsteroidal anti-inflammatory drugs. This complication usually develops in locations where the esophageal lumen is compressed by other anatomic structures.

Question 255

what category of cancer is this?

Answer 255

Squamous Cell Carcinoma

vs adenocarcinoma

Question 256

to what is the autoantibody in Grave’s dz directed

Answer 256

thyrotropin receptor antibodies (TRAb), which bind and activate the TSH receptor.

vs Hashimoto’s thyroiditis (hypothyroidism) in which the antibody is directed against thyroid peroxidase

Question 258

the hemolytic anemias are _____-cytic

Answer 258

normocytic

Question 259

what 2 major pathways cause normocytic anemia

Answer 259

low production of RBCs or increased desctruction

Question 260

the following are examples of what type of anemia?

hereditary spherocytosis

G6PD deficiency

Sickle Cell anemia

Answer 260

intrinsic hemolytic anemia

*by definintion, if its hemolytic, its normocytic

Question 261

pathophys of hereditary spherocytosis

Answer 261

Question 262

Young patient presents with fatigue and the attached lab values. whats the most likely diagnosis?

Answer 262

hemolytic anemia (probably one of the hereditary types)

Question 263

causes of aplastic crisis

Answer 263

Question 264

what microscopic finding is shown and what does it indicate?

Answer 264

Howell Jolly Bodies

• Some RBCs leave marrow with nuclear remnants
• Normally cleared by spleen
• Presence in peripheral blood indicates splenic dysfunction

Classic finding: spherocytes and Howell-Jolly bodies: Indicates patient post-splenectomy for spherocytosis

Question 265

pathophysiology of PNH (paroxysmal nocturnal hemoglobinuria)

Answer 265

PNH is caused by lack of the glycosylphosphatidylinositol (GPI) anchor protein that holds CD55 and CD59 proteins on the surface of erythrocytes. CD55 and CD59 prevent complement activation. Without these proteins, the complement system attacks red cells and causes hemolysis. This is most evident in the morning when urine is especially concentrated and turns red from the presence of heme.

Although erythrocytes are most at risk for complement damage, leukocytes and platelets are also vulnerable. This may result in pancytopenia. Other features of PNH include thrombosis (the most common cause of death), abdominal pain, fatigue, jaundice, and erectile dysfunction in men

Question 266

Schistocytes are seen in what type of hemolytic anemia?

Answer 266

with microangiopathic hemolytic anemia (e.g., HUS, TTP).

Question 267

Acrocyanosis - what is it?

Answer 267

Acrocyanosis is blue discoloration of the distal extremities, nose, and ears. This is seen in cold (IgM) hemolytic anemia.

Question 268

Flow cytometry for a patient shows deficiency of glycosylphosphatidylinositol (GPI)

what is the likely dx?

Answer 268

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare etiology of hemolytic anemia caused by lack of the glycosylphosphatidylinositol (GPI) anchor proteins that hold CD55 and CD59 on the surface of erythrocytes. CD55 and CD59 prevent complement activation. Without these proteins, the complement system attacks red cells and causes hemolysis and hemolytic anemia. Diagnosis can be made by flow cytometry showing a deficiency of GPI-anchored proteins.

Question 269

True or False: Elevated LDH is a lab finding common to all hemolytic anemias

Answer 269

LDH is released from red cells when hemolysis occurs. Patients with any cause of hemolytic anemia (including PNH) have elevated LDH levels. With less hemolysis, LDH levels decline.

n.b In clinical trials of eculizumab, LDH levels significantly decreased in all treated patients due to a decrease in hemolysis. LDH levels are commonly monitored as a marker of hemolysis in clinical trials.

Question 270

the microcytic anemias are

Answer 270

1. iron deficiency
2. anemia of chronic disease
3. thalassemia
4. sideroblastic anemia (which includes lead poisoning)

Question 271

what lab findings help differentiate between anemia of chronic disease and iron deficiency anemia

Answer 271

Question 272

dx of lead poisoning - what labs?

Answer 272

Question 273

What microscopic finding is shown and what type of anemia is it associated with

Answer 273

ringed sideroblast –> sideroblastic anemia

Question 274

what microcopic finding is shown and what disease do you commonly find it in

Answer 274

target cell in beta thallasemia

Question 275

what is HRI kinase?

Answer 275

heme-regulated inhibitor kinase

when active, it phosphorylates and therefore inactivates the transcription factor needed for globin synthesis. So heme is low, the HRI kinase is active, transcription factor is inactivated –> low globin synthesis

Question 276

1st physiological response to vessel (endothelial) damage

Answer 276

Endothelin (ET-1) is released from damaged cells and binds to receptors on the target tissue (e.g., adjacent vascular smooth muscle). ET receptors are coupled to a Gq-protein and the formation of IP3. Increased IP3 causes calcium release by the sarcoplasmic reticulum, which causes smooth muscle contraction.

ET-1 formation and release are stimulated by angiotensin II (AII), antidiuretic hormone (ADH), thrombin, cytokines, reactive oxygen species, and shearing forces acting on the vascular endothelium. ET-1 release is inhibited by prostacyclin and atrial natriuretic peptide as well as by nitric oxide.

Question 277

what is the round structure found in this histo slide of thymus tissue

Answer 277

Hassals corpuscle –> dense collection of thymic epithelial cells

Question 278

T/F: beta-globin is required for normal gestation

Answer 278

FALSE. since b globin synthesis starts late, it is not required (gamma-globin predominates until shortly after birth)

Question 279

what are the splenic sinusoids

Answer 279

Question 280

reticulocytes vs erythrocytes in blood smear

Answer 280

r=reticulocyte

Question 281

bone marrow histo

Answer 281

Question 282

Dual antiplatelet therapy is standard practice in patients with acute coronary syndromes (e.g., unstable angina, myocardial infarction) who receive stents. This therapy involves aspirin plus what?

Answer 282

P2Y12 antagonist such as clopidogrel or prasugrel.

Question 283

Phosphodiesterase inhibitors such as dipyridamole or cilostazol are used as vasodilators in the treatment of __________

Answer 283

claudication (cilostazol) or in pharmacologic stress testing (dipyridamole).

Question 284

what classic abnormal morphology is shown

Answer 284

Reed-Sternburg Cells (RS cells) –> enlarged B cells with mirror image nucleoli

classic finding in hodgkin lymphoma

Question 285

what classic abnormal morphology is shown

Answer 285

Reed-Sternburg Cells (RS cells) –> enlarged B cells with mirror image nucleoli

classic finding in hodgkin lymphoma

Question 286

what is the likely cancer diagnosis?

Answer 286

Burkitt Lymphoma (a type of Non-hodgkin lymphoma)

Question 287

what is the likely cancer diagnosis?

Answer 287

Burkitt Lymphoma (a type of Non-hodgkin lymphoma)

Question 288

Question 289

Question 290

what characteristic RBC pathology is shown and what disease is it associated with?

Answer 290

Rouleaux (like a “roll” of coins) formation –> assoc with MM d/t elevated levels of plasma proteins

Question 291

what characteristic RBC pathology is shown and what disease is it associated with?

Answer 291

Rouleaux (like a “roll” of coins) formation –> assoc with MM d/t elevated levels of plasma proteins

Question 292

what characteristic pathology is shown

Answer 292

Auer rods–> ch/o Acute pro-myelocytic leukemia (APL) (subtype of Acute Myeloid Leukemia)

Question 293

what characteristic pathology is shown

Answer 293

Auer rods–> ch/o Acute pro-myelocytic leukemia (APL) (subtype of Acute Myeloid Leukemia)

Question 294

T/F: medulla contains mostly mature T cells.

Answer 294

true

Bone marrow–derived dendritic cells are present at the corticomedullary junction and within the medulla, and macrophages are present primarily within the medulla. The migration of thymocytes through this anatomic arrangement allows physical interactions between the thymocytes and these other cells that are necessary for the maturation and selection of the T lymphocytes.

Functional and phenotypic differentiation into CD4 + CD8 − or CD8 + CD4 − SP T cells occurs in the medulla, and mature T cells are released into the circulation

So, by the time they get to the center of the medulla, they are almost all mature

Question 295

what are all the components released by platelet granules

Answer 295

ADP, TXA2, vWF, Serotonin –> recruit/activate more plts.

Calcium –> to be used in secondary hemostasis

Question 296

MOA of Warfarin

Answer 296

warfarin inhibits hepatic vitamin K epoxide reductase –> blocking synthesis (recycling) of the active, reduced form of vitamin K –> decreased γ-carboxylation of glutamate residues on coagulation factors II, VII, IX, and X –> reduced coagulation

Question 297

MOA of Heparin

Answer 297

Heparin binds to and potentiates the effects of antithrombin III, which inactivates thrombin and inhibits the coagulation factors II, VII, IX, X, XI, and XII.

Fast onset of action –> best for acute management

Question 298

what are the DOACs (direct oral anticoagulants) and for whom are they indicated and contraindicated?

Answer 298

1. Direct thrombin inhibitor: Dabigatran: reversibly binds to the active site on the thrombin molecule, preventing thrombin-mediated activation of coagulation factors
2. Factor Xa inhibitor: Apixaban, Rivaroxaban, Edoxaban (mn: Xa BANned)

They are indicated for patients with non-valvular AFIB.

Contraindicated for pts with valvular AFIB, pregnancy, or renal impairment

Question 299

AE of heparin

Answer 299

heparin-induced thrombocytopenia (paradoxical thrombosis in setting of low platelets –> most commonly in hospital setting)

hyperkalemia (MOA; toxic effect on adrenals –> hypoaldosteronism –> decreased aldosterone causes decreased K+ excretion [because aldosterone upregulates Na reabsorption in exchange for excreting K+])

osteoporosis

Question 300

T or F: Vitamin K does not affect platelet number

Answer 300

TRUE, so vit K deficiency could cause increase bleeding but not thrombocytopenia

Question 301

What are the vitamin K dependent clotting factors?

Answer 301

2, 7, 9, 10

Question 302

Indications for warfarin

Answer 302

prophylaxis of DVT, PE, AFIB-related thrombus

contraindicated during pregnancy –> bone problems