test #9 3.23 Flashcards Preview

UWorld mixed > test #9 3.23 > Flashcards

Flashcards in test #9 3.23 Deck (153)
Loading flashcards...
1
Q

which antidepressant is good for (1) depression w/ psychomotor retardation or hypersomnia (2) depression w/ smoking cessation desire (3) w/o sexual dysfxn

A

buproprion (NDRI and nAch-agonist). has stimulatory effects.

2
Q

which antidepressant is good for patients w/ insomnia. side effect?

A

trazodone (highly sedating antidepressant, but can cause priapism & other sexual side effects. SARI – 5-HT antagonist and reuptake inhibitor.

3
Q

SSRI-induced sexual dysfxn in what %age? what 3 types?

A

50%. decreased libido, anorgasmia, and increased latency to ejaculation.

4
Q

electrolyte abnormalities w/ hyperaldosteronism

A

hypokalemia, metabolic alkalosis. normal Na+ bc of aldosterone escape

5
Q

describe aldosterone escape

A

hypernatremia is not seen w/ hyperaldosteronism. increased Na+ reabsorption –> intravascular hypervolemia –> promote ANP release –> diuresis –> compensatory Na+ loss.

6
Q

presentation of primary mineralcorticoid excess

A

HTN, hypokalemia, suppressed renin, non-suppressible aldosterone

7
Q

normal serum Na+, hypokalemia, metabolic acidosis suggests..

A

renal tubular acidosis

8
Q

renal tubular acidosis types (1, 2, and 4)

A

1: distal: failure of H+ secretion by alpha-intercalated cells (hypokalemia) 2: proximal: failed HCO3- reabsorption in PCT 4: adrenal: aldosterone deficiency or resistance

9
Q

renal tubular acidosis explanation

A

inability to acidify urine

10
Q

how do CAG repeats in huntingtin gene lead to disease

A

code for abnormal huntingtin protein that decreases expression of OTHER genes by inhibiting transcription (hypermethylation of histone fragments). SILENCE of other neurotrophic genes

11
Q

mutation of homeodomain gene would lead to..

A

alteration in body structure // spontaneous abortion

12
Q

is macrocephaly a typical feature of common chromosomal syndromes

A

no

13
Q

cleft palate, polydactylyl, rocker-bottom feet associated w/

A

trisomy 13 (patau syndrome)

14
Q

down’s syndrome have increased risk of..

A

AML-M7 and ALL in childhood. Alzheimer’s in adulthood

15
Q

common presentation of trisomy 21 (5)

A

mental retardation, facial dysmorphism, single palmar crease, endocardial cushion defects, duodenal atresia

16
Q

case-control

A

select those w/ disease & w/o disease. then look back to look at exposure. calculate odds ratio

17
Q

controls in a case-control experiment

A

individuals w/o disease (w/ and w/o exposure). goal: exposure frequency among non-disease general population

18
Q

carcinoid heart disease (associated w/ carcinoid syndrome)

A

fibrous intimal thickening w/ endocardial plaques limited to the RIGHT heart (bc both 5-HT and bradykinin in blood are inactivated distally by pulmonary vascular endothelial monoamine oxidase). can result in pulmonic stenosis and restrictive cardiomyopathy.

19
Q

carcinoid syndrome symptoms. results from?

A

skin flushing, abdominal cramping, nausea, vomiting, diarrhea. results from production of serotonin, kallikrein, bradykinin, histamine, prostaglandins and/or tachykinins produced by carcinoid tumor.

20
Q

degree of endocardial fibrosis seen in carcinoid heart disease correlates w/..

A

plasma levels of serotonin and urinary excretion of 5-HIAA (5-hydroxyindoleacetic acid)

21
Q

elevated plasma levels of homocysteine associated w/..

A

arterial and venous thrombosis. also atheroscerlosis

22
Q

common way to injur PCL

A

MVA

23
Q

lateral vs. medial meniscus

A

lateral – rounder and covers a larger portion of the articular surface

24
Q

lateral vs. medial articular surface of knee size

A

medial articular surface is larger, as it bears more body weight

25
Q

conversion disorder

A

characterized by neurological symptoms (nonepileptic seizures, blindness, loss of sensation, weakness, paralysis) that are anatomically or physiologically inconsistent w/ neurologic disease but are NOT FEIGNED.

26
Q

2 most important mechanisms involved in the development of complications w/ diabetes

A

(1) advanced glycosylation end products (2) polyol pathway impairment

27
Q

describe how advanced glycosylation end products –> diabetic complications (microangiopathy, neuropathy, atheroscerlosis)

A

attach glucose to amino acids (reversible –> irreversible), accumulate & cross-link w/ collagen in blood vessel walls & interstitial tissue (MICROANGIOPATHY and NEUROPATHY). cross-linking also facilitates inflammatory cell invasion & deposition of LDL in vascular walls –> ATHEROSCLEROSIS

28
Q

describe how polyol pathway impairment –> diabetic complications (cataracts & peripheral neuropathy)

A

in tissues that do not depend on insulin for glucose transport (lens, peripheral nerves, blood vessels, kidney). glucose –> sorbitol (via aldose reductase). sorbitol –> fructose. both sorbitol & fructose increase osmotic pressure in tissue –> osmotic cellular injury. increased water in lens fiber cells –> rupture of cells –> opacification of lens and CATARACT formation. osmotic injury of Schwann cells –> PERIPHERAL NEUROPATHY

29
Q

which main tissues do not rely on insulin for glucose transport

A

lens, peripheral nerves, blood vessels, and kidney

30
Q

why do patients w/ galactosemia get cataracts?

A

galactose –> galactitol (via aldose reductase) –> osmotic damage of lens cells

31
Q

silent mutation

A

code for same amino acid

32
Q

missense

A

conservative: diff amino acid, but similar. nonconversative: diff amino acid entirely

33
Q

nonsense

A

premature stop codon

34
Q

short-acting benzodiazepine (2)

A

triazolam, alprazolam (<6 hours)

35
Q

intermediate acting benzodiazepine (1)

A

lorazepam (6-24 hours)

36
Q

long-acting benzodiazepine (3)

A

diazepam, flurazepam, chlordiazepoxide ( > 24 hours)

37
Q

if patient has increasing anxiety before SSRI effects kick-in (as it takes 4 wks).. you can..

A

give a benzodiazepine (choose short, intermediate, long)

38
Q

benzodiazepine of choice for person in mission-critical position?

A

triazolam, short short-acting

39
Q

triad of congenital toxo

A

hydrocephalus, intracranial calcifications, and chorioretinitis (inflamm of choroids and retina that can leave cotton-like white/yellow scars on retina)

40
Q

when can toxoplasma be transmitted in utero

A

only if mother infected during first 6 months of pregnancy.

41
Q

common complication of prematurity is?

A

intraventricular hemmorhage (can lead to long-term neurodevelopmental impairment) almost always occurs in infants born before 32 wks and/or birthweight <1500g. within first 5 postnatal days

42
Q

presentation of intraventricular hemorrhage in infant (3 levels of severity)

A

either (1) silent (2) altered consciousness, hypotonia, decreased spontaneous movt (3) bulging anterior fontanelle, hypotension, decebrate posteruring, tonic-clonic sz, irregular respiration, coma

43
Q

where does intraventricular hemorrhage in infant originate?

A

germinal matrix: highly cellular and vascularized layer in subventricular zone (from which neurons and glial cells migrate out during brain development). less prominent in 24-32 wks.

44
Q

describe the germinal matrix

A

in ventricles, contains numerous thin-walled vessels lacking glial fibers that support other blood vessels throughout the brain (contribute to intraventricular hemorrhage) becomes less prominent in 24-32 wks of gestation (reducing risk of IVH)

45
Q

shaking baby syndrome causes

A

tear of cortical bridging vein. subdural hematoma

46
Q

epidural hematoma

A

head trauma, tear middle meningeal artery. usu associated w/ temporal bone fracture

47
Q

PE results in…

A

acute pulmonary V/Q imbalance –> hypoxemia –> hyperventilation –> respiratory alkalosis

48
Q

LPS structure (3 regions)

A

very long, heat-stable molecule. 3 regions: O antigen, core polysaccharide, lipid A. lipid A –> toxic properties.

49
Q

how is LPS released into circulation

A

released during division or bacteriolysis. NOT ACTIVELY SECRETED>

50
Q

septic shock associated w/ systemic release of which signals

A

IL-1 and TNF-alpha (fever, hypotension, diarrhea, oligouria, vascular compromise, and DIC)

51
Q

what innervates the posterior wall of the external auditory canal? remainder external portion? internal portion?

A

posterior wall external auditory canal – Vagus (small auricular branch)

remainder of external auditory canal including the external portion of the tympanic membrane – Trigeminal (mandibular division) via auriculotemporal branch.

inner surface of TM –> glossopharyngeal nerve (tympanic branch)

52
Q

cutaneous sensation from vagus nerve?

A

to posterior external auditory canal via small auricular branch. (rest of canal is CN V3)

53
Q

vasovagal syncopal episode during otoscope exam?

A

touch posterior wall of external auditory meatus, decrease BP and HR

54
Q

chorda tympani carries (2)

A

(1) afferent taste fibers from anterior 2/3 of tongue (2) efferent parasympathetic preganglionic fibers to submandibular and sublingual glands

55
Q

anatomical landmark of left ventricle

A

fifth left intercostal space at left midclavicular line. covered by LUNGS. to penetrate left ventricle, would need to be DEEP. all other heart structures lie left to midclavicular line

56
Q

anatomical landmark of right ventricle

A

forms anterior (sternal) surface of heart, and majority of inferior border. lies left of midclavicular line

57
Q

t/f: lung overlies much of the anterior surface of heart

A

TRUE

58
Q

first line treatment for atrial fibrillation w/ rapid ventricular response (AF w/ RVR)? second line?

A

first line: diltiazem, verapamil and B-blockers. second line: digoxin, bc it slows conduction through AV node (via vagal stimulation)

59
Q

delayed afterdepolarizations. what drug can trigger this?

A

can occur after complete repolarization of the cardiac myocytes in states of hyperexcitability (i.e. very high intracellular Ca++ or high catecholamine stimulation states). digoxin toxicity can lead to this (as it increases intracellular Ca++)

60
Q

what does decreased AP duration lead to?

A

decreased refractory period –> allow cardiac pacemaker cells to fire / conduct AP w/ greater frequency.

61
Q

the shorter the refractory period of atria..

A

the more frequently the cells can depolarize (predispose to atrial fibrillation)

62
Q

what are the blood pressure limits in diabetics?

A

normal blood pressure limits LOWER.

63
Q

normal loss of albumin in urine? microalbuminuria?

A

normal loss: 20mg/day or less. microalbuminuria: loss of 30-300mg/day. indicates diabetic nephropathy.

64
Q

what level of protein concentration can be detected by urinalysis

A

MACROalbuminuria: greater than 300

note: microalbuminuria 30-300mg/day

65
Q

name 3 alpha-1-blockers (HTN and BPH)

A

doxazosin, prazosin, terazosin

66
Q

drug of choice for HTN in patients w/ coronary artery disease & CHF?

A

cardioselective beta-blockers like metoprolol and atenolol

67
Q

drug of choice for essential HTN in general population, osteoperosis, and isolated systolic HTN?

A

hydrochlorothiazide

68
Q

isosorbide dinitrate

A

rx: stable angina pectoris. decrease CO by venodilitation –> decrease preload.

69
Q

almost half of all UTIs in sexually active young women caused by

A

staphylococcus saphrophyticus (resistant to novobiocin & coag neg)

70
Q

which bacterial species produces DNAse?

A

group A strep. degrades DNA in pus to facilitate spread of organism. (can use antiDNAse test in pts w/ strep infxn and glomerulonephritis)

71
Q

cushing disease (excess ACTH) results in hyperplasia of..

A

adrenocortical zona fasiculata and reticularis

72
Q

(1) acute-onset CVP >15 cm H20 (central venous pressure, as demonstrated by increased JVD) and (2) hypotension w/ tachycardia indicates (3) pulsus paradoxus

A

cardiac tamponade or tension pneumothorax

73
Q

cause of cardiac tamponade

A

serous viral pericarditis and significant acute pericardiac effusion

74
Q

beck’s triad of cardiac tamponade

A

(1) hypotension (2) distended neck veins (3) distant/muffled heart sounds on auscultation, as well as tachycardia. also, pulsus paradoxus

75
Q

acute fibrinous pericarditis follows? causes?

A

follow an URI. causes pleuritic chest pain and pericardial friction rub

76
Q

constrictive pericarditis

A

chronic process that requires months-> years to produce constriction sufficient to cause tamponade. not acute!

77
Q

maple syrup urine disease

A

defective breakdown of branched chain amino acids (leucine, isoleucine, and valine). defective branched chain alpha-keto acid dehydrogenase. build up of alpha-keto acid in tissue and serum –> neurotoxicity

78
Q

when does maple syrup urine disease manifest? rx?

A

usu first few days of life. urine –> burned caramel. can be life threatening. rx: dietary restriction of branched-chain amino acids

79
Q

hypermethioninemia

A

benign disorder, defective metabolism of methionine by enzyme methionine adenosyltransferase.

80
Q

where is PAH secreted into nephron lumen?

A

proximal tubule. lowest conc of PAH in luminal fluid is bowman’s space

81
Q

most common congenital anomalies seen in women and men are..

A

accessory nipples (polythelia). usu asymptomatic, but can swell or become tender along w/ other breast tissue. and become symptomatic in pregnancy.

82
Q

how does polythelial occur? where do they occur?

A

accessory nipples. failure of appropriate involution of mammary ridge. can occur anywhere along embryonic milk line at lower chest and abdomen, and are bilateral in 50% of patients.

83
Q

what are ephelides

A

freckles. hyperpigmented, usu not raised. small and found on sun-exposed areas. don’t change w/ hormone

84
Q

keloid

A

raised and can be pigmented. occur as a result of scarring from some type of trauma to skin. not influenced by menstrual cycle

85
Q

lipomas

A

benign fatty tumors felt under skin. no hyperpigmentation. not influenced by mentstrual cycle hormone changes

86
Q

cutaneous neurofibromas are? what do they contain?

A

benign, soft, flesh-coloured, or lightly pigmented skin nodules. consisting of schwann cells and neural fibroblasts.

87
Q

button-hole sign of neurofibroma

A

applying pressure to neurofibroma can cause them to retract into subcutaneous tissue.

88
Q

the most common skin lesion

A

nevi, often confused w/ accessory nipple. can be raised and hyperpigmented. not influenced by hormonal changes.

89
Q

jaw claudication // chewing difficulty

A

most specific symptom of giant cell (temporal) arteritis. granulomatous inflammation of the media. tend to be > 50y/o

90
Q

medial granulomas are seen in which vasculitides?

A

(1) giant cell / temporal arteritis (2) takayasu arteritis

91
Q

takayasu arteritis

A

usu affects aortic arch (w/ remainder of aorta and its branches in 1/3 of pts) medial granuloma. usu females < 40.

92
Q

distinction between dian cell lesions of aorta is often made on..

A

patient’s age. > 50 = giant cell (temporal) arteritis. < 40 y/o female = takayasu arteritis.

93
Q

leukocytoclastic vasculitis = microscopic polyangiitis, microscopic polyarteritis, hypersensitivity vasculitis

A

segmental fibrinoid necrosis of small vessels. histologically similar to polyarteritis nodosa

94
Q

polyarteritis nodosa

A

segmental transmural necrotizing inflammation of medium to small-sized arteries.

95
Q

thromboangiitis obliterans (buerger’s disease)

A

thrombosing vasculitis of medium and small sized vasculature (principally tibial and radial artery). most commonly seen in heavy smokers w/ onset prior to 35 y/o

96
Q

rheumatoid arteritis

A

hypersensitivty vasculitis affecting arterioles and arteries of any size. visceral infarctions after long-standing rheumatoid arthritis. pathology = similar to polyarteritis. diff from giant cell.

97
Q

immunity to reinfection w/ influenza type A (and likely B and C) mediated by..

A

predominately by specific anti-hemagglutinin IgA and IgG antibodies

98
Q

rx: for acute decompensated congestive heart hailure?

A

aim to reduce volume overload. LOOP DIURETICS. maximum diuresis in shortest test.

99
Q

3 common uses for furosemide, toresmide, bumetanide, ethacrynic acid

A

(1) pulmonary edema (2) venous and pulmonary congestion secondary to CHF (3) peripheral edema

100
Q

common and less common side effects of loop diueretics

A

common: hypokalemia, hypomagnesemia, hypocalcemia. less common: volume depletion, hponatremia, decreased GFR, hypotension, OTOtoxicity

101
Q

who secretes von willebrand factor? what is it attached to?

A

endothelial cells; circualtes in multimers that are noncovalently attached to factor VIII (increases its stability)

102
Q

DDAVP causes..

A

increases vWF secretion from endothelial cells (increase in 30-60min). useful in mild vWD. is similar to vasopressin but not identical

103
Q

how is DDAVP unlike vassopressin.

A

DDAVP has only minimal effect on V1 vasopressin receptor (therefore, little vasoconstriction)

104
Q

DDAVP in hemophilia A?

A

increases vWF, increases stability of factor VIII

105
Q

where are most clotting factors formed. what is the exception

A

most clotting factors = liver. vWF = endothelial cells.

106
Q

treatment of enuresis

A

DDAVP tablets

107
Q

definition of orthostatic hypotension

A

fall of >20mm in systolic or >10mm in diastolic BP when upright. standing causes pooling of significant amount of blood in veins (500-1000ml)

108
Q

list 4 ways standing alters hemodynamics

A

(1) decreased venous return to heart (2) decreased ventricular filling and subsequent drop in CO and BP (3) drop in BP stimulates compensatory baroreceptor reflex (4) increased sympathetic tone raises peripheral vascular resistance through alpta-1-adrenoreceptors –> increased venous return and cardiac output

109
Q

what does active antithrombin III do?

A

binds factor Xa and prevents it from converting prothrombin –> thrombin

110
Q

partial pressure of oxygen at which 50% of hemoglobin are saturated (P50)? myoglobin

A

hemoglobin P50: 26mmHg. myoglobin p50: 1mmHg; has only 1 heme group.

111
Q

individual subunits of hemoglobin

A

structurally analogous to myoblohin

112
Q

hemodynamic actions of epinephrine on 1. HR 2. systolic BP 3. diastolic BP & associated receptors

A

(1) HR increases, B1 (2) systolic BP increases, B1 and A1 (3) diastolic BP decreases at LOW dose (B2 > A1); increases at HIGH dose (A1 > B2)

effect on diastolic BP depends on dose! low –> B2. high –> A1

113
Q

phenlyephrine

A

selective alpha-adrenergic agonist. elevated systolic and diastolic BP, may cause reflex bradycardia.

114
Q

phentolamine

A

nonspecific alpha1 and alpha 2 blocker (no beta effect). causes fall in diastolic BP and reflex tachycardia.

115
Q

isoproterenol

A

nonspecific beta agonist. vasodilation (B2) and increased HR and contractility (B1)

116
Q

paroxysmal supraventricular tachycardia caused by…? Rx?

A

typically results from re-entrant impulse traveling through slowly and rapidly conducting segments of AV node. rx: increase cardiac parasympathetic tone –> carotid sinus massage and valsalva. or adenosine in hospital

117
Q

how does parasympathetic nervous system slow heart rate?

A

primarily by slowing conduction through AV node

118
Q

autosomal dominant polycystic kidney disease pathologic features

A

mutations in PKD-1 or PKD-2 –> tubular cell proliferation & secretion. any point in nephron, but <5% nephrons affected. microscopic cysts at birth, enlarge over decades. compress renal parenchyma – atrophy & fibrosis

119
Q

autosomal dominant polycystic kidney disease clinical features

A

freq clinically SILENT, 50% undiagnosed. flank pain, hematuria, HTN. renal failure slowly over 10-20 yrs. ESRD by 70. extrarenal manifestations (liver cysts, cerebral aneursyms)

120
Q

multicystic kidney dysplasia

A

characterized by multiple cysts of varying size in kidney and ABSENCE OF NORMAL PELVOCALICEAL system. condition associated w/ ureteral or ureteropelvic atresia. affected kidney –> nonfxnl. abdominal ultrasound of fetus -> diagnostic.

121
Q

Auer rods are highly suggestive of..

A

AML, esp M1 M2 M3, but can be any type.

122
Q

AML diagnostic criteria

A

> 20% myeloblasts in peripheral blood or marrow. usu adults, median age 65

123
Q

presentation of hairy cell leukemia

A

splenomegaly, cytopenia, circulating hairy cells (small-medium lymphoid cells w/ hairy projections)

124
Q

why do infants maintain babinski up to 12 months?

A

incomplete myelination of corticospinal tracts

125
Q

positive straight leg test

A

pain elicited in supine patient when leg held straight while raised off examining table – sign of sciatic nerve root (L4-S3) irritation. sensitive for intervertebral disc hernation –> sciatica

126
Q

how commonly is medullary thyroid cancer familial

A

20% (part of MEN type 2A&B) or familiary medullary thyroid cancer syndrome

127
Q

familial medullary thyroid cancer often have

A

germ-line mutations in RET proto-oncogene.

128
Q

medullary thyroid cancer arises from..

A

parafollicular calcitonin-secreting C-cells. uniform polygonal or spindle-shaped cells w/ extracellular amyloid deposits. amyloid stains w/ congo Red

129
Q

papillary thyroid cancer

A

papillary structure w/ calcifications (psammoma bodies) and large, ground glass, grooved nuclei.

130
Q

PSAMMOMA mnemonic

A

papillary carcinoma of thyroid, serous cystadenocarcinoma, meningioma, and mesothelioma.

131
Q

large pleomorphic cells in thyroid..

A

anaplastic thyroid carcinoma, poor prognosis.

132
Q

collagen subtype 1 & associated disease

A

bone, ligament, skin (dermis), dentin, cornea, blood vessels, SCAR tissue. associated disease: osteogenesis imperfecta

133
Q

collagen subtype 2

A

cartilage, vitreous humor & nucleus pulposus

134
Q

collagen subtype 3 & associated disease

A

reticular fibers: skin, lungs, intestines, blood vessels, bone marrow, lymphatics & granulation tissue. associated w/ ehlers-danlos syndrome (type 3 & 4)

135
Q

collagen subtype 4 & associated disease

A

basement membrane; associated w/ alport syndrome

136
Q

evolution of myocardial infarction: (a) 0-4hr (b) 4-12hr (c) 12-24hr (d) 1-5d (e) 5-10d (f) 10-14d (g) 2wk-2mo

A

(a) 0-4hr: minimal change.
(b) 4-12hr: early coagulation necrosis, edema, hemorrhage, wavy fibers
(c) 12-24hr: coagulation necrosis & marginal contraction band necrosis
(d) 1-5day: coagulation necrosis & neutrophilic infiltrate
(e) 5-10day: macrophage phagocytosis of dead cells
(f) 10-14day: granulation tissue & neovascularization
(g) 2wk-2mo: collagen deposition & scar formation

137
Q

p27

A

cell cycle inhibitor. acts during G1 by inhibiting CDKs. normal tissue have high levels of p27. malignant tissues have low p27

138
Q

retinoblastoma regulation of G1-S transition

A

active (hypophosphorylated) and inactive (hyperphosphorylated). resting cells (G0) have active Rb. when growth factor stimulation, activation of cyclin D, cyclin E, and *CDK 4 (upreg in response to signal) and 6 occurs, and Rb is hyperphosphorylated (inactivates)–> releases E2F transcription factor. allows progress through G1->S

139
Q

Ras

A

part of MAP-kinase cascade. not associated w/ G1-S transition

140
Q

important CYP inducers

A

carbamazepine, phenobarb, pheytoin, rifampin, griseofulvin

141
Q

important CYP inhibitors

A

cimetidine, ciprofloxacin, erythromycin, azole antifungals, gradefruit juice, isoniazid, ritonavir (protease inhibitors)

142
Q

general note re: oral antibiotics and drug interaction w/ warfarin

A

oral antibiotics reduce intestinal bacteria load, reduces vitamin K synthesis, could potentiate warfarin’s anticoagulant effects

143
Q

structure of collagen molecule

A

3 polypeptide alpha chains held together by H-bonds (rope-like triple helix)

144
Q

Ehlers-Danlos clinical manifestation & pathophys

A

group of rare heriditary disorders involving defect in synthesis of collagen found in skin, tendons, ligaments, and muscles. manifests as over-flexible joints, over-elastic skin, and fragile tissue susceptible to bruising, wounding, and hemarthrosis.

145
Q

common mutations leading to ehlers-danlos (2)

A

either deficiencies of (1) lysyl-hydroxylase or (2) pro-collagen peptidase enzymes responsible for collagen synthesis

146
Q

fibrillin-1 is present in? defects related to

A

major component of microfibrils that form sheath around elastin. abundant in blood vessels and suspensory ligaments of lens. defects in fibrillin-1 –> autosomal dominant marfans

147
Q

laminins

A

heterotrimeric glycoproteins that bind to type IV collagen underlying epithelial cells (organization and fxn of basement membrane)

148
Q

fibronectin

A

binds to integrins on cell surface and components of ECM (i.e. collagen and fibrin). fxns as adhesive protein; involved in cellular differentiation, phagocytosis, platelet adhesion, thrombus formation

149
Q

keratin

A

major component of hair and skin (in epidermal cells, makes outmost layer of skin waterproof). defects in keratin 5 and 14 –> epidermolysis bullosa simplex

150
Q

elastin

A

fibrous protein in CT, can be stretched but will recoil. synthesized from polypeptide precusor tropoelastin. in skin, blood vessels, and lung alveoli

151
Q

atrophy

A

loss in cell substance that results in reduced cell size or death. physiologic –> early in develop: regress

152
Q

physiologic atrophy

A

regression of notochord & thyroglossal duct in fetus. reduction in maternal uterine size after childbirth.

153
Q

pathologic atrophy

A

usu in response to something: decreased physical workload, loss of innervation, decreased blood supply, inadequate nutrition, absent endocrine stimulation, aging, pressure