uworld assessment block #2 4.15 Flashcards Preview

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Flashcards in uworld assessment block #2 4.15 Deck (90):
1

depletion of B12 stores? folate?

B12 depletes over years.

folate affected (by alcohol abuse) within weeks

2

MS: Lhermitte's phenomenon

shock-like sensation radiating to the feet with neck flexion. also have cognitive defects.

B12 deficiency does this too.

3

guillain-barre syndrome presentation

acute postinfetious polyneuropathy. nonspecific prodome and nausea followed by progressive ascending paralysis.

4

sensory stroke presentation

numbness of face, arm, leg on one side of body w/o accompanying motor defect

5

pathophysiology of myasthenia gravis

acquired autoimmune condition. fluctuating weakness of facial, periocular, bulbar, pelvic girdle muscles.

antibody-mediated, T-cell dependent attach on postsynaptic NMJ.

6

myasthenia gravis is associated w/ what additional finding

60-70% have hyperplastic thymus tissue. 10-15% have thymomas.

removal of thymus helps.

7

loss of heterogeneity

inherit mutation in one tumor suppressor, and then develop somatic mutation later. allows for malignant transformation (i.e. Rb)

8

parental imprinting

preferential transcription of genes from one or the other homologous pair of chromosomes depending on parental origin of chromosome

9

common causative organisms for infective arthritis by age (children < 2, late adolescence & early adult, older children/older adults, sickle cell.

-children <2: H. flu
-late adolescence & early adult: N. gonorrhea (esp women)
-older children/adult: s. aureus
-sickle: salmonella

10

third pharyngeal arch

stylopharyngeus muscle, hyoid, glossopharyngeal n.

11

fourth pharyngeal arch

intrinsic muscles of pharynx + cricothyroid

CN X: superior laryngeal n.

12

hyperkalemia in DKA? hyponatremia

1. acidemia drives K+H+ exchange
2. insulin tends to drive K+ into cells
3. note: total body K+ low bc osmotic diuresis and GI loss.

hyponatremia:
1. osmotic activity of glucose (serum Na drops 1.6 mEq/L for every 100mg/dl rise in glucose)
2. hyperglycemia-induced osmotic diuresis

13

arnold chiari malformation

downward displacement of elongated cerebellar tonsils through foramen magnum & into upper cervical canal. CSF flow blocked.

additional findings: small posterior fossa, caudal displacement of medulla, hydrocephalus, lumbar myelomeningocele

14

dandy-walker malformation

characterized by enlarged posterior fossa.

absent/shrunken vermis replaced by large midline cyst = expanded fourth ventricle

15

spina bifida cystica (myelomeningocele)

herniation of a sac containing CSF, spinal cord, nerve roots through defect in vertebral arches and dura

16

syringobulbia

fluid-filled cavities within medulla oblongata

17

inheritance of androgen insensitivity (testicular feminization syndrome)

rare X-linked disorder. 46 XY.

absence of testosterone receptors in target tissue.

testosterone and LH high (lack of receptors prevents negative feedback)

circulating androgens eventually peripherally converted to estradiol -- feminization.

no mullerian duct (MIF from sertoli) so vagina = blind duct

testes: abdomen, inguinal canal, labia majora. make testosterone, but no spermatogenesis

18

normal testes descent

-transabdominal: MIF
-inguinoscrotal: hCG and androgens

19

mullerian agenesis

uterus, fallopian tubes, proximal vaginal fail to develop. LH, FSH, estrogen normal.

cause of primary ammenorrhea

20

when does the germinal matrix subside in prominence

after 32 weeks. decline in cellularity & vascularity.

21

why is germinal matrix prone to hemorrhage?

highly cellular & vascular (generates neurons & glia during fetal development).

no network fibers present to support vasculature in germinal matrix.

22

cephalhematoma

hemorrhage within circular region of scalp that inters the lower uterus during childbirth.

associated with sudden delivery, disproportion between diameter of fetal head & birth canal, inappropriate forceps use.

23

cisplatin toxicity

ototoxicity. damages apical stereocilia on hair cells

24

amiodarone toxicity in lungs? other toxicity?

insterstitial pneumonitis

dyspnea, cough, dry inspiratory crackles, patchy interstitial infiltration on radiograph.

other: hepatic necrosis, thyroid abnormalities, corneal deposits, bluish-gray skin discoloration

25

adverse effects of sotalol

TdT and excessive beta blockade

26

procainamide toxicity

drug-induced SLE (anti-histone)

27

prostaglandin PGE1

stimulate gastric epithelial cell mucus production & decrease parietal cell acid secretion

28

misoprostol for GI

PGE1 analog.

stimulate gastric epithelial cell mucus production.

may also decrease parietal cell acid secretion )

29

submandibular lymphadenopathy suggests

head & neck cancer: vast majority: SQUAMOUS CELL CARCINOMA. associated w/ tobacco and alcohol.

develop in oral cavity: ventral tongue, floor of mouth, lower lip, soft palate, gingiva.

often infiltrates adjacent tissue.

30

nitroglycerin effect on heart

venodilation predominantely.
reduced preload --> decreased effective circulating blood flow due to venous pooling.

cardiac reflex compensates for drop in SV, CO, MAP w/ sympathetic increase in HR and contractility.

new steady state: decreased preload, afterload, end systolic ventricular volume, CO. TPR relatively unchanged.

overall, myocardial consumption decreased --> relieved angina / ischemia

31

Liddle syndrome

pseudohyperaldosteronism.

ENaC overexpression in CT

32

CFTR in sweat glands?

reabsorb Cl- and enhance Na+ resorption.

defect: excessive Cl- and Na+ in sweat

33

CFTR in respiratory and intestinal epithelium

active secretion Cl-.

defect: no Cl- secretion, increased Na+ resorption. passive H20 resorption

34

albright hereditary osteodystrophy (AHO)

autosomal DOMINANT

end organ resistance to PTH

pseudohypoparathyroidism.

skeletal and developmental defects (short stature, short metacarpal and metatarsal bones). end-organ resistance to PTH, TSH, and LH/FSH. (all G-s-alpha-mediated pathways)

35

allelic heterogeneity vs genetic heterogeneity vs. phenotypic heterogeneity

allelic heterogeneity: DIFFERENT mutations
SAME gene locus
SIMILAR phenotypes.
(i.e. complete loss of fxn vs. partial loss of protein)

genetic heterogeneity: mutations of DIFFERENT genes SIMILAR phenotypes

phenotypic heterogeneity: mutations in SAME gene
DIFFERENT phenotype

36

duchenne vs. becker

duchenne: complete loss of fxn in dystrophin

becker: structurally abnormal but partially fxn dystrophin.

37

inheritance of type II DM

polygenic

38

hemochromatosis

autosomal recessive.

excessive GI absorption of iron.

hemosiderin in dermis and various parenchymal organs.

silent until at least 20 grams accumulated.

hepatomegaly, skin hyperpigmentation (hemosiderin deposit in dermis, DM (pancreatic islet destruction), impotency, arthropathy, cardiac dysfunction & enlargement.

elevated plasma iron w/ >50% saturation of transferrin, elevated ferritin.

39

defects in melanocyte proliferation & migration

Piebaldism (partial albininism) and Waardenburg syndrome.

blue-gray macules referred to Mongolian spots, arise when melanocytes are halted in dermis as they migrate from neural crest to epidermis.

40

Menke's disease

abnormally pigmented, kinky hair, hypopigmented irises.

41

latex agglutination

detect for presence of antigen.

add sample to collection of specific of antibodies fixed to latex beads

42

erythema with yellow "honey" crush on cutaneous wound is.. caused by?

IMPETIGO.

most common cause: s. aureus and group A strep.

note: s. aureus can lead to bullous impetigo (toxin production)

43

s. epidermis?

normal skin flora. not commonly associated w/ skin disease. regarded as contaminant if found in wound culture.

44

hypoparathyroidism sign

Chvostek sign: tap cheek (facial n) --> contract facial muscle
Trousseau sign: take BP (occlusion of brachial artery, cuff Triceps) --> carpal spasm.

tetany due to hypocalcemia (due to accidental surgical excision of parathyroid glands, autoimmune destruction or DiGeorge

45

drug effux pumps often use

derivive energy from H+ gradient or Na+ gradient or direct ATP

46

achondroplasia mutation

point mutation FGFR3 (substitute Arg for Gly) in 375 position, chr 4p.

increased function. severely restricted chondrocyte proliferation in the growth plate.

47

mutation in marfan's

fibrillin-1, chromosome 15q

48

mutation in osteogenesis imperfecta

COL1A1 chr 17q
COL1A2 chr.7q

49

ewing's sarcoma translocation

t(11,22) joins EWS gene from chr 22 to FLI1 gene

50

brutton's agammaglobulinemia

X-linked. defect BTK. poor maturation of B cells, can't leave bone marrow. no CD19 in circulation.

51

chr 7 diseases

cystic fibrosis, ehlers-danlos, osteogenesis imperfecta

52

chr 16 diseases

polycystic kidney disease, tuberous sclerosis

53

signal sequences for synthesis into ER

N-terminal HYDROPHOBIC

54

maple syrup urine disease

defective branched chain alpha-keto acid dehydrogenase complex.

can't degrade leucine, isoleucine, and valine --> CNS toxicity.

life threatening if untreated.

rx: early restriction of isoleucine, leucine, valine.

normally:
valine, isoleucine ---> prionyl-CoA
leuine ---> acetyl-CoA
via branched-chain alpha-keto acid dehydrogenase

55

hydrops fetalis with thalassemia

Hb Barts, severe form of alpha thalassemia characterized by four nonfunctional alpha globin loci

microcytic, hypochromic RBCs. occasional target cells.

56

high concentration of local testosterone

maintained by androgen-binding protein (ABP) within the seminiferous tubule. made by sertoli cells.

makes them less lipophillic, concentrating them within the luminal fluid.

57

glucagon signalling via

Gs protein
adencylate cyclase (in hepatocytes, myocardiocytes, and adipocytes)
increased cAMP

activate glycogen phosphorylase

like B2 -- Gs

glycogenolysis and gluconeogenesis in hepatocytes.

58

intracellular mediator for growth factor & insulin

intrinsic tyrosine kinase activity

59

Ras is related to..

MAP kinase cascade -- control of cell ENTRY into CELL CYCLE

60

alpha 1 and V1 work on..

Gq, PLC, IP3

61

sympathetic innervation of sweat glands in axilla

T2 sympathetic ganglion

62

superior cervical sympathetic ganglion provides sympathetic innervation to...

tarsal muscle, lacrimal gland, radial muscle of iris, and all salivary glands

63

greater, lesser, least splanchnics

sympathetic preganglion neurons into abdomen, synapse on postganglionic cell bodies within celiac and aorticorenal ganglia (sympathetics to abdominal viscera)

can also pass to adrenal medulla without synapsing.

64

vagus nerve parasympatics

all parasympathetic preganglion fibers to viscera of thorax, foregut, midgut.

65

red safranin O stain

colours cartilage, mast cell granules, and mucin red

66

dystrophin location

cytoplasmic! intracellular anchor

67

round ligament of the uterus

derivative of gubernaculum.

connects uterus fundus to labia majora THROUGH (round) inguinal canal

68

broad ligament

wide fold of peritoneum that connects uterus to pelvis side wall.

contains ovaries, fallopian tubes, round ligament of uterus & uterus

69

infundibulopelvic ligament (suspensory ligament of the ovaries

connects ovaries to lateral pelvic wall. carries ovarian vessels.

70

cardinal ligament

connects cervix to side wall of pelvis, contains uterine vessels

(ureters at risk of injury during ligation of uterine vessels)

71

ovarian ligament

medial pole of ovary to lateral uterus

latches to lateral uterus

72

conversion of NE to E?

conversion of E to metanephrine?

metanephrine to vanillylmandelic acid?

NE --> E
phenylethanolamine-N-methyl transferase (PNMT) uses S-adenosyl methionine (SAM)

E --> metanephrine
catechol-O-methyltransferase

metanephrine --> VMA
MAO

73

COMT is important for

degradation of epinephrine to metanephrine

74

MAO is important for

degradation of metanephrine to VMA

75

rate limiting step in catecholamine synthesis

tyrosine to DOPA (tyrosine hydroxylase)

76

DOPA decarboxylase requires what cofactor

vitamin B6

77

primary respiratory issue with PE

hypoxemia secondary to pulmonary V/Q mistmatch.

hypoxemia --> hyperventillation and respiratory alkalosis.

increased pH, reduced PaO2 and PaCO2.

78

primary respiratory issue with COPD

slightly decrease pH, increased PaCO2, decreased PaO2.

79

platelet activating factor

phospholipid inflammatory mediator. causes severe bronchoconstriction, vasoconstrition, and platelet aggregation (with microthrombus formation).

low dose -> relatedd to NO production with eNOS. vasodilator, promotes leukocyte adhesion to endothelium, diapedesis (transmigration), degranulation, and oxidative bursts.

made by platelets, basopphils, mast cells, neutrophils, monocytes, macrophages, and endothelial cells.

Gq -> PLC -> IP3 -> calcium

80

prostacyclin signalling

Gs: adenylate cyclase, cAMP.

inhibit platelet function

81

thromboxane and PAF signalling

Gq --> PLC, IP3, Ca2+

activate platelets

82

cilastozol and dipyrimidole

PDE III inhibitor, increase cAMP in cells --> vasodilation and inhibit platemet activation

83

which agents directly promote healing of peptic ulcer

bismuth subsalicylate & sucralfate

84

which agents directly promote symptom relief of peptic ulcer

calcium carbonate, proton pump inhibitors, H2 blockers

85

why are antibiotics used in PUD?

treat disease recurrence.

86

2 or more of the following required for diagnosis of NF1 (chr 17)

6+ cafe au lait spots, intertriginous freckling, 2+ cutaneous neurofibromas or 1 plexiform neurofibroma, optic nerve glioma, characteristic bony lesions, iris Lisch nodules, 1st degree relative

CA NN OT FAI L2 B 1st

cafe au lait
2+ neurofibromas
optic glioma
freckling of axilla or inguinal
2 Lisch nodules

bone abnormality: kyphoscoliosis, tibia dysplasia (bowing), sphenoid dysplasia

1st degree relative

neurofibromas = benign growth of schwann cells

87

epidermoid cysts

sebaceous cysts, keratin-filling, cystic papules, central punctum. not actually from sebaceous glands

88

what side of heart do IV drug users mess up?

right side! tricuspid and pulmonary

89

important adverse reaction to vancomycin

- "red main" syndrome
- nonspecific mast cell degranulation when vanc is infused too rapidly
- NOT an IgE mediated reaction

increases with RATE of infusion.

90

pencillin G adverse reaction

IgE mediated hypersensitivity reaction. not directly related to RATE of infusion.