General patho 0421Q Flashcards

1
Q

Congenital heart defect with down syndrome

A

endocardial cushion defects: ostium primum ASD, regurgitant AV valves

ostium primum ASD: cleft in anterior leaflet of mitral valve AND septal leaflet of tricuspid valve

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2
Q

Congenital heart defect with DiGeorge

A

TOF and aortic arch anomalies

other features: thymic aplasia, failure of parathyroid formation

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3
Q

Congenital heart defect with Friedrich’s ataxia

A

hypertrophic cardiomyopathy

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4
Q

Congenital heart defect with Marfan

A

cystic medial necrosis of aorta

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5
Q

Congenital heart defect with Tuberous sclerosis

A

valvular obstruction due to CARDIAC RHABDOMYOMA

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6
Q

Congenital heart defect with turner’s syndrome

A

coarctation of aorta

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7
Q

Heart abnormality with Kartageners syndrome

A

situs inversus

other features: recurrent sinusitis, infertility, bronchiectasis - all due to immotile cilia from microtubular dynein arm defect

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8
Q

What is the final phase of acute tubular necrosis?

A

3 phases: inciting event, maintenance, recovery

RECOVERY PHASE
polyuric - pt can become dehydrated and develop severe hypokalemia due to high volume, hypotonic urine

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9
Q

What changes are seen with hyperkalemia?

A

above 6.0 mEq/L
peaked T waves on EKG
potentially fatal vent arrhythmias

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10
Q

patho change seen with CIRRHOSIS

A

diffuse hepatic fibrosis with nodular parenchymal regeneration.

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11
Q

patho change seen with primary biliary cirrhosis

A

granulomatous destruction of bile ducts

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12
Q

What is Guillain barre syndrome?

A

acute demyelinating peripheral neuropathy.
affects young adults. preceded by febrile illness (esp. campylobacter). Abs against infectious Ags cross-react with myelin of spinal roots/peripheral nn. present with ascending paralysis.
endoneural inflammatory infiltrate.

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13
Q

How does diphtheria affect nerves?

A

toxin penetration of blood-nerve barrier leading to peripheral neuropathy

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14
Q

What is pure red cell aplasia?

A

marrow failure with severe hypoplasia of marrow erythroid elements in setting of normal granulopoiesis and thrombopoiesis [normal WBC and plt count]

assoc. with thymoma, lymphocytic leukemias, parvovirus B19

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15
Q

What are EPO-producing conditions?

A
renal cell carcinoma
hepatocellular carcinoma
cerebellar hemangioblastoma
uterine fibroids
etc...

[cause polycythemia]

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16
Q

What is chronic pancreatitis?

A

diarrhea, weight loss, epigastric region calcifications in chronic alcoholic.
leads to panc exocrine insuff (due to ductal obstruction causing atrophy of acinar cells and panc fibrosis) and malabsorption.

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17
Q

What is shaken baby syndrome?

A

subdural hematoma.
bilateral retinal hemorrhages.
immature brain moves within skull, leading to tearing of bridging veins

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18
Q

What intracranial change is assoc. with skull fracture?

A

middle meningeal artery tear.

epidural hematoma.

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19
Q

embolic stroke

A

embolism: Afib w/o h/o anticoag tx.
acute hypoxic or complete ischemic injury leads to coagulative necrosis in most organs BUT LIQUEFACTIVE NECROSIS IN BRAIN (irreversible) - infarcted brain tissue replaced by cystic astroglial scar

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20
Q

N-myc oncogene

A

overexpressed in neuroblastoma and small cell of lung

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21
Q

MS

A

optic neuritis: pain with ocular mvmt, decreased vision.

cerebellar dysfunction, i.e. intention tremor

22
Q

diseases assoc with carpal tunnel syndrome

A
*repetitive wrist mvmts
fluid retention: hypothyroidism, renal failure, pregnancy
DM
acromegaly
rheumatoid arthritis
dialysis-assoc. amyloidosis
23
Q

craniopharyngioma

A

tumor arising from remant of Rathke’s pouch.

3 components: solid (actual tumor cells); cystic (filled with machinery oil liquid); calcified

24
Q

most frequent tumor in immunosuppressed pts

A

primary CNS lymphoma - dense cellular aggregates of uniform, atypical lymphoid cells - most often from B cells.
assoc. with EBV

25
Q

mediators that attract/activate neutrophils

A
  1. LTB4
  2. C5a
  3. IL-8
  4. bact products
26
Q

what 2 things mediate pain?

A
  1. PGE2

2. bradykinin

27
Q

most common cause of congenital prolonged QT

A

Jervell and Lange-Nielsen syndrome - accompanied by neurosensory deafness

28
Q

what enzyme is released by injured hepatocytes and bacteria, leading to brown pigment stone formation?

A

beta-glucuronidase - hydrolysis of bilirubin glucuronides, increased unconj bili in bile. related to Ecoli, ascaris, and liver fluke (O.sinensis) infx

29
Q

what is the most common cause of calcium kidney stone dz?

A

idiopathic hypercalciuria: normal serum calcium with high urine calcium excretion

30
Q

what is the most common cause of chronic renal failure?

A

diabetes mellitus

31
Q

what is strongly assoc. with BLUE SCLERA?

A

osteogenesis imperfecta - defective synthesis of collagen type 1

32
Q

what is decreased in Alzheimers?

A

ACh in nucleus basalis of Meynert and hippocampus - caused by decreased activity of choline acetyltransferase (CHAT)

clinical ex: progressive memory loss with diffuse cortical and hippocampal atrophy on MRI

33
Q

what is the major function of the hippocampus?

A

formation of new memories

34
Q

when do cluster HAs occur?

A

during sleep, behind one eye, 15-90 min long

assoc. with lacrimation, nasal congestion, ptosis. more common in males.

35
Q

what gene mutations are assoc. with hereditary breast cancer?

A

BRCA 1 and 2 - tumor suppressor genes needed for gene repair and regulation of cell cycle. increased risk of breast and ovarian cancer. AD inheritance. chromo 17.

36
Q

axonal reaction

A

changes seen in neuronal body after axon is severed - cell body shows sign of edema (swelling, rounding. nuc displaced to periphery). Nissl substance becomes fine, granular, and dispersed throughout cytoplasm (central chromatolysis).

visible 24-48 hrs after injury.
reflect increased prot synthesis in order to repair axon.

37
Q

what changes are typical of compression atrophy?

A

decrease in size and number of neurons due to increased intracranial pressure or mass lesion

38
Q

what does BACK PAIN not relieved by rest or position change indicate?

A

neoplastic BONE disease

39
Q

what does metastatic prostate cancer have a strong predilection for?

A

bones, esp. axial skeleton (blastic/sclerotic effect) - increased alk phos, radionuclide bone scan

40
Q

how does HCTZ affect serum calcium levels?

A

increased distal tubular reabsorption of filtered Ca – increased serum Ca suppresses PTH

41
Q

what does the combo of recurrent resp infxs and panc insuff in a child suggest?

A

CYSTIC FIBROSIS

42
Q

what are the clin manifestations of vit E deficiency?

A

hemolytic anemia. neuromuscular disease: skel myopathy, spinocerebellar ataxia, pigmented retinopathy

43
Q

what triad is assoc. with paroxysmal nocturnal hemoglobinuria (PNH)?

A
  1. hemolytic anemia
  2. hypercoag state (thrombosis, esp. venous)
  3. decreased bld counts

*due to CD55 and 59 deficiency that disallows inactivation of complement

44
Q

which lung cancer is assoc. with nonsmokers and females?

A

adenocarcinoma (bronchial)

45
Q

what organ systems are involved with ankylosing spondylitis?

A
  1. MSS: peripheral enthesitis (inflamm. at sites of tendon insertion)
  2. resp: enthesopathy of costovertebral and costosternal junctions (limited chest wall expansion leading to hypoventilation)
  3. cardio: ascending aortitis leading to dilation of aortic ring and aortic insuff (AR)
  4. eye: anterior uveitis with pain, blurred vision, photophobia, conjunctival erythema
46
Q

what is perianal fistula assoc. with?

A

Crohns disease (involves full thickness of bowel wall) - insidious development with alternating constipation and nonbloody diarrhea

47
Q

what are black pigment stones assoc. with?

A

chronic extravascular hemolysis

ex: sickle cell, beta thal, hereditary spherocytosis - increased unconj bili

48
Q

what are the main components of black pigment stones?

A

calcium carbonates and phosphates (most are radiopaque)

49
Q

what type of gallstones does obesity predispose to?

A

cholesterol gallstones

50
Q

what seronegative spondyloarthropathies are related to HLA-B27?

A

Psoriatic arthritis
Ankylosing spondylitis
IBD-assoc. spondylitis
Reiter syndrome (Reactive arthritis)