test #21 4.11 Flashcards Preview

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Flashcards in test #21 4.11 Deck (114):
1

tumors associated w/ von-Hippel Lindau

hemangioblastomas, pheochromocytoma, and renal cell carcinoma

2

mutations in retinoblastoma associated with..

osteosarcoma and bilateral retinoblastoma (Rb - tumor suppressor involved in transition of G1 to S)

3

location of MEN1 mutation

MENIN gene chromosome 11

4

which MEN syndrome is associated with marfanoid habitus

MEN2B

5

sign of mature fetal lungs in amniocentesis

lecithin / sphingomyelin ratio > or = 2.

lecithin = phoshatidylcholine.
mature = making surfactant

6

in suspected erythroblastosis fetalis, measure amniotic fluid for..

billirubin

7

amniotic measurement of 17-hydroxyprogesterone tests for..

21-B hydroxlase deficiency

8

main form of therapy for urea cycle (ornithine cycle) defects?

protein restriction (enough for growth & development though)

9

heterozygotes for sickle cell trait (HbAS) are..

protected from sickle cell crises, aplastic crises, sequestration crises (bc >50% HbA).

protection from Plasmodium falciparum.

peripheral smears are usu normal, as is retic count.

WILL sickle when sodium metabisulfite is added

10

increased MCHC

mean corpuscular hemoglobin concentration. increased when dehydrated.

11

nasal mucosal ulcerations & glomerulonephritis

wegener's (granulomatosis w/ polyangiitis)

12

c-ANCA + is virtually pathognomonic for..

Wegener's (granulomatosis w/ polyangiitis). target neutrophil proteinase 3

13

smooth muscle cell antibodies..

autoimmune hepatitis

14

antimitochondrial antibodies

primary biliary cirrhosis

15

expectations by age 3

parallel play (not cooperative), tricycle, climb stairs, identify self w/ name and gender, copy shapes.

16

most common cause of aseptic meningitis?

eneterovirus (90%) of cases. fecal-oral (coxsackie, echo, polio) replicate in GI tract but usu don't cause gastroenteritis.

17

presentation of poliomyelitis

unvaccinated immigrant from endemic regions.

fever, malaise, aseptic meningitis first ((photophobia, painful extraocular muscle movements)

then myalgia and asymmetric paralysis (classically affecting legs) -- damage to lower motor neurons -- HYPOREFLEXIC paralysis

18

physiostigmine

rx: atropine overdose. tertiary amine, so crosses blood brain barrier (fixes PNS and CNS). acetylcholinesterase inhibitor

19

which artery runs with radian nerve?

DEEP brachial artery.

brachial artery travels medially along arm into antecubital fossa.

20

midshaft fracture of humerus (2)

radian n. & deep brachial artery damage

21

non-extensor actions of radian n. (2)

brachioradiali (forearm flexor) also supinator muscles

22

supracondylar fractures of humerus (2)

proximal median n. & brachial artery

23

periodic acid-schiff reaction

periodic acid oxidizes carbon-carbon bonds --> forms aldehydes that product magenta when react w/ fuschin-sulfurous acid.

good at detecting polysaccharides, mucosubstances, basement membranes.

diastatse + PAS = glycogen (will digest glycogen to maltose and glucose, which will wash off, negative rxn)

24

strongly PAS+ intestine

whipple disease (infxn w. tropheryma whipplei)

25

describe malabsorption & symptoms of whipple disease

infection w/ the actinomycete tropheryma whipplei in macrophages of small intestine--> foamy macrophages --> compress lacteals and impair fat absorption. PAS+ & diastase-resistant intestine.

foamy whipped -- CANN
cardiac, arthralgia, mesenteric nodes, neurological symptoms.

usu older men.

26

calcium colour on H&E

dark purple

27

iron stain

prussian blue, dark blue

28

neutral lipid stain

nile red or sudan black

29

path of CN III

courses between PCA and SCA as it leaves midbrain. susceptible to injury from aneurysm in these vessels.

travels w/ PComm.

30

aneurysms in PComm, PCA, or SCA can affect

oculomotor n.

31

aneurysms in AICA can compress (2)

facial and vestibulocochlear n.

32

2 ways loop diuretics (furosemide) stimulate diuresis

1. block NKCC (limit Na+ Cl- reabsorption
2. stimulate prostaglandin release (vasodilate afferent arteriole, increasing GFR)

33

Nesiritide

BNP analog, used in setting of acute decompensated heart failure for (1) vasodilation & (2) diuretic effects

34

broca aphasia have problems w/ writing/signing?

YES. damage to inferior frontal gyrus of dominant (usu left) impairs all communicative motor planning!

35

associated impairment w/ broca aphasia

right hemiparesis

36

associated impairment w/ wernicke aphasia

right superior visual field defect

37

describe problems w/ conduction aphasia

lesion of arcuate fasiculus. fluent speech w/ phonemic errors. preserved comprehension but poor repetition.

38

damage to frontal eye fields cause..

deviation to ipsilateral side.

located immediately anterior to precentral gyrus

39

calories / gram of (1) protein (2) carbs (3) alcohol (4)

(1) protein: 4 cal/gram (2) carbohydrates: 4cal/gram (3) alcohol: 7cal/gram (4) fat: 9cal/gram

40

transcortical aphasias. (motor, sensory, mixed)

maintain ability to repeat.

transcortical motor: repeat, nonfluent speech, intact comprehension.

transcortical sensory: repeat, fluent, poor comprehension

transcortical mixed: repeat, poor comprehension, nonfluent speech.

41

extensive lymphocytic infiltration and granulomatous destruction of interlobular bile ducts

primary biliary cirrhosis (autoimmune destruction of intrahepatic bile ducts and cholestasis). "florid duct lesion:

most common in middle age women, insiduous onset. pruitis, fatigue, hepatosplenomegaly, evidence of cholestasis (xanthelasma, pale stool)

42

middle age women w/ fever, prolonged episode of severe RUQ pain after fatty meal

acute cholescystitis

43

man w/ long history of ulcerative colitis w/ fatigue and high ALP?

primary sclerosing cholangitis.

unknown cause: concentric "onion-skin" bile duct fibrosis --> alternating strictures and dilation with beading of intra & extrahepatic bile ducts on ERCP

44

cholangitis vs. cholecystitis

cholangitis: bacterial infection of BILE DUCT. usu ascending w/ gram - bacteria. presents as sepsis (high fever, chills) jaundice, abdominal pain. increased incidence of choledocholithiasis

cholecystitis: acute or chronic inflammation of GALLBLADDER. usu from cholelithiasis. (rarely primary w/ CMV). will also have increased ALP if bile ducts become involved

45

what causes cholecystitis?

usu secondary to obstruction: cholesthiasis. RARELY primary (CMV).

46

Rokitansky-Aschoff sign of chronic cholescystitis

herniation of gallbladder mucosa into muscle wall. Rokitansky-Aschoff sinus -- look like ducts in muscle. late complication: porcelain gallbladder.

47

3 clinical presentation of Fabry disease? enzyme deficiency? build-up metabolite? inheritance

X-linked recessive. defecient alpha-galactosidase-A. build up ceramide trihexoside.
1. peripheral neuropathy (hands/feet)
2. angiokeratoma
3. cardiovascular / renal disease

earliest signs: hypohidrosis, acroparaesthesia (episodic, debilitating, burning neuropathic pain), angiokeratoma (punctate, dark red, non-blanching macules & papules between umbillicus and knees)

die from renal failure

48

fucosidosis

rare lysosomal disease, deficiency of alpha-L-fucosidase. can have spasticity.

49

pts at risk for acute leukemia

down syndrome, patau syndrome, ataxia-telangiectasia, bloom syndrome, fanconi anemia

50

degree of cyanosis in tetrology of fallot

depends on degree of pulmonic stenosis (determines degree of right-to-left shunting)

51

clinical presentation of legionella pneumonia? X-ray?

HYPONATREMIA. pneumonia. watery diarrhea. headache & confusion. sputum w/ neutrophils but no organism.

exposure to contaminated water (cruise or hotel).

unlike OTHER atypical pneumonias -- usu see unilobal infiltrate that progresses to consolidation

52

etiology of hyponatremia in legionella

inappropriate release of ADH and/or renal tubulointerstitial disease that impairs reabsorption of Na+.

53

DNA Pol III

5'-3' synthesis w/ 3-5' exonuclease activity (proofread while synthesizing, can mix a mistake it just made)

54

DNA Pol I

5-3' exonuclease activity w/ 5'-3' synthesis too

55

cause of instability // hypervariation in hepatitis C RNA-dependent-RNA pol

no 3'-5' exonuclease (proofreading) activity

56

reaction triggered by thymidylate synthase. what does it also require?

dUMP ---> dTMP, gets methyl group from N5,N10 methylene tetrahydrofolate --> DHF.

DHF then ---> THF by dihydrofolate reductase (inhibited by methotrexate)

57

DNA intercalaters (causing defective base pairing and splitting of DNA strands)

anthracyclines (daunorubicin and doxorubicin)

58

integrase inhibitor

raltegravir

59

describe acanthosis nigricans

hyperkeratotic, hyperpigmented plaques w/ velvety texture. skin tags (acrochordons) are pedunculated outgrowths of normal skin that are commonly present

60

benign acanthosis nigricans? malignant?

benign: insulin resistance (increased levels of insulin & insulin-like growth factors --> epidermal and dermal proliferation) like diabetes mellitus, acromegaly, obesity

malignant: underlying neoplasm. GI and GU tract. gastric adenocarcinoma most especially.

61

K+ regulation in kidney

100% filtered, 65%reabsorbed PCT, 35% reabsorbed in TAL.

MAJOR mediations in principal and alpha-intercalated cells.

hypokalemia: reabsorption via H/K+ ATPase on a-intercalated cells.

hyperkalemia: secretion via K+ channels on principal cells

62

4 states promoting K+ excretion

1. high extracellular K+ levels
2. increased aldosterone
3. alkalosis
4. increased fluid flow through tubules (i.e. volume expansion, high sodium intake, diuretic use)

63

types of AML

m0-m7. m3 is APML, associated with t(15,17).

64

3 most common causes of hair loss in adults between 18-45

(1) male pattern baldness (androgenetic alopecia)
(2) tinea capitis (ringworm)
(3) telogen effluvium

only first is inherited (polygenic or autosomal dominant

65

hair loss w/ male pattern baldness vs. tinea capitis

tinea capitis: isolate pruritic lesions on scalp. central clearing. rx: terbinafine (squalene epoxide inhibitor). need SYSTEMIC bc topical do not penetrate hair follicle enough

male pattern: receding hairline & patch at vertex

66

alopecia areata

autoimmune disorder that causes patchy/diffuse hair loss. rx: topical corticosteroids

67

rx for severe acne

oral isotretinoin. inhibits follicular epidermal keratinization (maintains specialized epithlium)

68

cholestasis can lead to what vitamin deficiency? what may result?

vitamin ADEK!! fat soluble. can have osteomalacia!

69

both obstructive and nonobstructive cholestasis characterized by..

deposition of bile pigment within the hepatic parenchyma. green-brown plugs in dilated bile canaliculi. (impaired absorption of vitamin ADEK)

70

half-life

(0.7Vd) / Cl

71

maintenance dose

amount that must be administered to equal amount that is cleared

= (desired plasma concentration x Cl)/bioavailability fraction

72

loading dose

(Vd x desired plasma concentration) / bioavailability fraction

73

when patients have renal / hepatic failure, what pharmacokinetic parameter must be altered?

maintenance dose, bc clearance will be altered.

74

mesenteric adenitis

children 5-14y/o. yersenia enterocolitica. nonspecific, abdominal pain in right lower quadrant

75

late complication of appendicitis

peritonitis or abscess formation (not fistula)

76

main presentation of crohn's disease? ulcerative colitis

crohn's -- transmural -- abdominal pain.

ulcerative colitis -- mucosa -- bloody diarrhea

77

transtentorial herniation (uncal)

medial temporal lobe through crus cerebri and tentorium

78

subfalcine herniation

cingulate gyrus under falx cerebri -- compress AVA

79

tonsilar herniation

cerebellar tonsils through foramen magnum -- compress medulla

80

presentation of transtentorial herniation (4)

(1) ipsilateral oculomotor - ptosis & down & out
(2) PCA occlusion - contralateral homonymous hemianopsia
(3) contralateral crus cerebri - ipsilateral spastic paralysis -- "false localization sign"
(4) duret hemorrhage: brainstem hemorrhage in pons and midbrain due to stretching and rupture of basilar artery (fatal)

81

tetracyclines during pregnancy (2)

fetal bone growth retardation and discoloration of deciduous teeth

82

ACE inhibitor use in pregnancy

TERATOGEN

83

disorganized schizophrenia

incoherent speech, inappropriate affect, too disorganized to take care of self / perform daily activities of living (showering, dressing)

84

residual schizophrenia

persistence of symptoms like emotional blunting, eccentric behavior, loose associations between episodes of ACTIVE schizophrenia.

LACK prominent hallucinations, delusions, disorganized speech, catatonia. BURNED OUT schizophrenic

85

undifferentiated schizophrenia

schizophrenic but do not fall into subtype. no prominent feature.

86

which hepatitis virus carries increased risk of hepatocellular carcinoma?

hepatitis B, C, and D

87

most effective way to decline HCC worldwide?

HBV vaccination

88

rapid acting insulin (3). best when?

lispro, aspart, glulisine. best for post-meal hyperglycemia.

action less than 15min

89

short-acting insulin (1) best when?

normal insulin. best IV.

takes 30min to start work subcut, w/ peak in 2-4 hours. bc forms dimers and hexamers.

90

intermediate acting insulin (1)

NPH (twice daily--basal)

neutral protamine Hagedorn. crystalline suspension w/ protamine and zinc. prolongs activty by delaying absorption.

91

long-acting insulin (2)

glargine, determir. once daily

glargine: ppts in subcutaneous tissue -- very slow absorption
determir: fatty acid side-chain, allows albumin binding & slow dissolution

92

types of insulin needed for diabetes

often need a basal (long-acting) + postprandial (short-acting)

93

IgA protease made by..

Neisseria gonorrhea (genital region) and meningitidis (oropharynx)

94

presentation of epiglottitis

sore throat, DYSPHAGIA, DROOLING, respiratory distress. haemophilus influenzae

95

common etiological agents of nasopharyngitis (common cold)

nasal congestion, discharge, sneezing, cough, sore throat

rhinovirus, influenza virus, coronavirus

96

presentation of laryngotracheitis (croup)

parainfluenza virus

upper respiratory tract symptoms followed by hoarseness, BARKING COUGH, STRIDOR, and repsiratory distress

STEEPLE SIGN

97

bronchiolitis

upper respiratory tract symptoms followed by WHEEZING, cough, respiratory distress.

RSV

98

3 post-processing of pre-RNA or hnRNA

methylguanosine cap, poly-A tail, splicing

99

major site of digestion of dietary lipids? absorption.

DIGESTION: duodenum.
ABSORPTION: jejunum

100

describe fat absorption

bile salts, fatty acids, 2-monoacylglycerol, cholesterol form micelles. allows close contact w. gut. fatty acid, cholesterol, monoaceylglycerol diffuse through brush border membrane (no assistance from transporters).

inside cell: triglycerides & cholesteryl esters reconstructed -- chylomicrons.

101

bile acid reabsoprtion

in ileum. enterohepatic circulation

102

how does cholcystectomy affect fat absorption

increase in RATE of enterohepatic circulation (no storage for bile --> constantly released into duodenum.

less able to tolerate large fatty meals bc do not have ability to release large amount of stored bile in a coordinated fashion.

103

what can the stomach absorb

water and alcohol.

104

where are fat soluble vitamins absorbed

like lipids, in jejuneum

105

what is absorbed in ileum

B12 and bile salts

106

what is absorbed in colon

fluid and electrolytes

107

howell-jowel bodies

nuclear remnants in RBCs. sign of splenectomy / asplenia

108

where do we see spherocytes

autosomal dominant hereditary spherocytosis, autoimmune hemolytic anemia, burns, un-fresh blood samples

109

target cells seen in..

obstructive liver disease, thalassemia, iron deficiency anemia, asplenism

110

most common cause of sporadic encephalitis. macroscopic? histology?

HSV-1. temporal lobe. reactivation of latent virus "living" in trigeminal ganglion. virus' spread along this nerve into cerebral vault.

macrosopic: edema and hemorrhagic necrosis of temporal lobes. Cowdry type A intranuclear inclusions in glial and neuronal cells. multinucleated giant cells.

can DIE from it

111

first week of TB exposure

if droplets small enough to enter alveoli, can ESTABLISH infection. large droplets cleared w/ mucocilary motion.

mycobacteria phagocytosed by alveolar macrophage. sulfatide virulence factor allows for INTRACELLULAR PROLIFERATION.

later, cellular lysis. infect others. virulence factors recruit more inactivated neutrophils and undermine immunological signaling between them.

EVENTUALLY antigen carrying macrophages / dendritic cells go to lymph, induce helper T cell (2-4 wks later)

112

describe epitheliod transformation of macrophages

after activation of cell-mediated immune response (2-4 wks after exposure). Th1 activated macrophages consume undigestible ish (TB), loose motility, accumulate, transform into epitheliod cells. eventually -- multinucleated Langhans giant cells form -- fusion of multiple epitheliod cells. arranged in arc along periphery

113

scattered caseous necrosis in TB (GHON FOCI)

secondary immune response due to Th1 T lymphocyte activation of macrophages. release chemotactic cytokines (like IFN-gamma) to attract other leukocytes. enhanced inflammatory response against foci of TB. results in locate tissue necrosis, macrophages, giant cells, fibroblasts --> combine to form caseating granuloma. small foci of bacteria killed, while larger necrotic areas are WALLED off --> GHON FOCI.

ghon foci -- potential source of reactivation if immunosuppressed

114

B cell response to TB

after antigen presenting cell activates T cells, which do both Th1 and Th2 response. immunoglobulins are hardly helpful bc intracellular.