6/21 Flashcards

Question

pseudomonas is associated with what type of skin problem

Answer 1

ecthyma gangrenosum skin patches w/ necrosis 2/2 lack of blood flow

Answer 2

exotoxin A- protein synthesis inhibition elastase- degrades elastin / vessel destruction phospholipase C- degrades cellular membranes pyocyanin- generates ROS

Answer 3

"dancing eyes dancing feet" associated with childhood neuroblastoma- most common adrenal medulla tumor in children abdominal mass that CAN cross midline (vs Wilms tumor)

Answer 4

malignant bone tumor in teenagers, esp long bones may resemble acute osteomyelitis

Answer 5

nephroblastoma in young children presents w/ palpable flank mass micro looks like primitive metanephric tissue

Answer 6

Schwann cells sense axonal degeneration and begin to degrade their myelin, secrete cytokines and chemokine that recruit macrophages the clearance of myelin debris and trophic factor secretion stimulates growth cone formation from proximal axons tump to facilitate nerve regeneration

Answer 7

macrophages/microglia are recruited slowly (BBB). oligodendrocytes that normally produce myelin become inactive/apoptose. myelin debris removal is slowed (years) which suppresses axonal growth via myelin-associated inhibitory factors. astrocytes also release inhibitory molecs and proliferate after injury forming a GLIAL SCAR that acts as a barrier to axon regeneration Wallerian degeneration- axon degradation distal to site of injury

Answer 8

NADPH oxidase deficiency decreased ROS, decreased oxidative burst needed for neutrophils increased susceptibility to catalase positive organisms no green/blue on diagnostic imaging tests

Answer 9

decreased lung compliance- willingness to expand when a pressure is exerted

Answer 10

a blue-green heme-based enzyme that is released from neutrophil azurophilic granules in pus/sputum of bacterial infections it catalyzes chloride + H2O2--> bleach during phagocytic respiratory burst

Answer 11

immune deficiency that's usually asymptomatic w. recurrent sinopulmonary and GI infections and/or autoimmune disease. Can have anaphylaxis when given blood transfusion

Answer 12

poor leukocyte adherence and transmigration through vasculature recurrent skin/mucosal infections no pus formation delayed umbilical cord detachment persistent leukocytosis defect in LFA-1 ingetgrin on phagocytes CD18

Answer 13

stimulates differentiation of "naive" T-helper cells into Th1 deficiency in IL-12 receptors leads to mycobacterial infections 2/2 inability to mount strong cell-mediated granulomatous response tx w/ IFN-gamma

Answer 14

given identical surface tension, a smaller sphere will collapse before a larger one without any surfactant contribution because there has to be more distending pressure

Answer 15

both: synthesized in large polypeptide non-polar AAs collagen: many are hydroxylated form disulfide bridges and triple helixes elastin: few are hydroxylated Lysyl oxidase + Copper destroys cross-links involving lysine

Answer 16

``` small cell: SIADH- (ADH hyponatremia w/ euvolemia) Cushing (ACTH) Lambert-Eaton Cerebellar ataxia ``` Squamous cell: Hypercalcemia (PTHrP)

Answer 17

neuroendocrine cell- small cell carcinoma

Answer 18

affects small airways, causing bronchiolitis obliterates characterized by lymphocytic inflammation, fibrosis, and ultimately destruction of bronchioles

Answer 19

mucociliary clearance happens in terminal bronchioles distal to that, phagocytosis and alveolar macrophages use lysosomal degradation and pulm lymphatics goblet cells end just before bronchioles begin

Answer 20

squamous cell carcinoma commonly presents w/ compressing adjacent structures from its superior sulcus tumor location Horner syndrome: ptosis, miosis, anhidrosis, enophthalmos Pain in ulnar nerve distribution Superior Vena Cava syndrome (facial edema, JVD) erosion of adjacent vertebral structures

Answer 21

higher pO2 lower pCO2 higher pH

Answer 22

lower pO2 (92 vs 160) you increase your Hct to increase your O₂ carrying capacity PaO2 and SaO2 both remain low because they're independent of Hgb concentration Dissolved O₂ depends on pAO2 and solubility in blood -when pAO2 decreases (at high altitude), both paO2 and O₂ dissolved in blood decrease Bound O₂ depends on Hb saturation SaO2 is independent of Hct the degree of Hb O₂ saturation is determined by pAO2 -at sea level, Hb is close to fully saturated -at altitude, pAO2 and SaO2 decrease the increase in Hgb concentration allows you to carry normal O₂ amounts even though your pAO2 and SaO2 are decreased at altitude

Answer 23

sphenopalantine artery, a terminal branch of the maxillary artery, which comes from the external carotid artery

Answer 24

post-viral (Influenza) pt w/ salmon colored sputum cavitary lesion

Answer 25

``` Macrolides (except azithro) Quinidine, Quinolones Gemfibrozil Avirs Ciprofloxacin Acute Alcohol Grapefruit Juice Isoniazid (INH) Amiodarone Sulfonamides Ketoconazole Cimetidine ``` MacQuin and his friend GemAvir cip alcohol acutely w/ Grapefruit juice and Ise, then A-S-K for cimetidine for their stomach ache

Answer 26

``` Nevirapine Carbamazepine Rifampin Barbiturates Phenytoin St. John's Wort Modafinil Chronic alcohol use Griseofulvin ``` Nevir drive your CAR up the RAMP through the BARBwire PHENce into St. John's Woods. You'll mo-dafinitely be chronically attached by Griszley bears

Answer 27

pts w/ an allergy to sulfa drugs, for example trimethoprim/sulfamethoxazole

Answer 28

upper cavitary lesion in pts w/ encapsulated GN rods alcoholics, aspiration, and abscesses lactose fermenter, urease positive, immotile, currant red jelly sputum

Answer 29

internal carotid ophthalmic artery retinal artery painless unilateral blindness

Answer 30

umbilical vein is oxygenated umbilical arteries are deoxygenated ductus venosus bypasses portal circulation ductus arteriosus bypasses lungs

Answer 31

interstitial myocardial granulomas found in rheumatic fever 2/2 bacterial endocarditis also seen w/ Anitschkow cells- enlarged macrophages w/ ovoid, wavy, rodlike nuclei (like ribbons)

Answer 32

Atrial natriuretic peptide: released from atrial myocytes in response to high blood vol/A pressure increases cGMP--> vasodilation and decreased Na reabsorption at collecting tubule promotes diuresis via constricting efferent and dilating afferent arterioles contributes to "aldosterone escape" mech ``` BNP: brain released from ventricular myocytes in response to tension longer half life than ANP blood test to dx HF available as Nesiritide to tx HF ```

Answer 33

honeycomb lung, esp in subpleura spaces interstitial fibrosis w/ cystic air spaces dry cough

Answer 34

Relative = normal RBC mass 2/2 dehydration or excessive diuresis Absolute = true increase in RBC mass 2/2 polycythemia vera, where all 3 cell lines increase (with low EPO levels) (Primary erythrocytosis) or 2/2 secondary erythrocytosis, where EPO could be produced by tumors or stimulated via hypoxia; only RBCs increase (nl WBC and plts) (secondary)

Answer 35

fibroses inhibit cholesterol 7-alpha-hydroxylase, which is supposed to catalyze synthesis of bile acids. the jelly fish's main goal is to up regulate LPL (lighthouses) to improve triglyceride (trident-yielding) clearance, and decrease hepatic VLDL secretion. it actives PPAR-alpha to induce HDL synthesis main goal: lower TG reduced bile acid production results in decreased cholesterol solubility in bile and favors formation of cholesterol gallstones. big risk of gallstones and myopathies, esp with statins.

Answer 36

GLUT-4 is insulin DEPENDENT found on skeletal muscle and adipocytes all others are insulin-independent GLUT-1: RBCs, brain, cornea, placenta GLUT-2: beta islet cells, liver, kidney, SI GLUT-3: brain, placenta GLUT-5: spermatocytes, GI tract

Answer 37

there's a greater insulin response w/ oral glucose (vs IV) because of things like glucagon-like-peptide 1 (GLP1) that are released after meals from gut, independent of blood glucose level and increased beta cell sensitivity to glucose

Answer 38

``` methylmalonic acid (cofactor in Methylmalonyl CoA mutase --> Succinyl CoA) which causes impaired myelin synthesis ``` Homocysteine (cofactor in Methionine synthase --> methionine) which causes impaired DNA synthesis pt will present with: megaloblastic anemia and pancytopenia, both related to impaired DNA synthesis subacute combined degeneration of dorsal columns (loss of proprioception/vibration, Romberg sign) and lateral cortical spinal tract (spastic muscle weakness, hyperreflexia)

Answer 39

AKA pentose phosphate pathway occurs in cytoplasm provides NADPH from abundantly available Glucose-6-Phosphate no ATP is used/produced requires G6PD as its rate limiting step! sites: lactating mammary glands, liver, adrenal cortex (sites of fatty acid/sterol synthesis), RBCs the NADPH produced is necessary to keep glutathione reduced, which keeps free radicals and peroxides detoxified

Answer 40

it prevents NADPH production via G6P + (NADP+) without NADPH, you have free radical and peroxide toxicity free radicals will cause oxidative damage, particularly to RBCs, causing hemolytic anemia oxidative stress will also denature the Hgb, cause it to ppt, and form Heinz Bodies the Heinz bodies will be phagocytized via splenic macrophages, and create Bite Cells "Bite into some Heinz ketchup"

Answer 41

SNoW DRoP Southern Blot = DNA Northern Blot = RNA Western Blot = Proteins Southwestern Blot = DNA-binding proteins

Answer 42

DNA Pol 3: only on prokaryotes elongates leading strand and lagging strand uses 3'-->5' exonuclease to proofread DNA Pol 1: only on prokaryotes elongates leading strand and lagging strand ALSO uses 5'-->3' exonuclease to remove RNA primer and replace w/ DNA (via 5'-->3' polymerase activity) Primase: makes RNA primer Gyrase: AKA topoisomerase removes supercoils/tension Helicase: unzips DNA at template fork Ligase: combines Okazaki fragments via phosphodiester bonds

Answer 43

OTC deficiency: most common urea cycle deficiency -hyperammonemia (body cannot eliminate ammonia) -excess carbamoyl phosphate is shunted to orotic acid (pyrimidine synthesis pathway), so you have high elevated orotic acid -carbamoyl phosphate + ornithine normally participate in urea cycle to make urea, but ornithine needs OTC transport protein to get into the mitochondria to form citrulline, eventually producing urea -hyperammonemia!! -Tx w/ very low protein diet so you don't have excess NH3 formation Orotic aciduria: defect in UMP synthase causing: inability to convert orotic acid to UMP (in the de novo pyrimidine synthesis pathway) -MEGALOBLASTIC ANEMIA (2/2 impaired DNA synthesis) -orotic acid in urine -no hyperammonemia!!! -Tx w/ UMP to bypass mutated enzyme

Answer 44

Pellagra- "3 D's" dermatitis (sun exposed areas) diarrhea (GI atrophy) dementia (neuro degeneration) Vit B3 is essential for NAD Vit B3 can come via dietary intake or synthesized via tryptophan Pellagra can also be seen in carcinoid syndrome, prolonged INH therapy, or Hartnup disease (deficiency in neutral AA transporters)

Answer 45

deficient homogentisate oxidase in tyrosine --> fumarate to go to the TCA cycle causes blue-black pigment-forming homogentistic acid to accumulate in tissue, esp CT and sclerae! urine turns black in air "tyrosine --> fumarate" "black tires --> fumes"

Answer 46

excess homocysteine, intellectual disability, osteoporosis, marfanoid habitus, kyphosis, lens subluxation, THROMBOTIC EVENTS and atherosclerosis normally: Homocysteine --> either of 2: methionine via methionine synthase + B12 cystathionine then to cysteine via CBS + serine + B6 cysteine is now essential tx w/ Vit B6 to "force" CBS activity

Answer 47

polyol pathway: Glucose --> sorbitol via aldose reductase + NADPH sorbitol --> Fructose via Sorbitol dehydrogenase + NAD+ sorbitol cannot cross cell membranes insufficient sorbitol dehydrogenase causes sorbitol accumulation, which can cause cataracts, retinopathy, and peripheral neuropathy, esp in pts w/ chronic hyperglycemia/DM

Answer 48

bright red w/o cyanosis labs show lactic acidosis and narrowing of the venous-arterial PO2 gradient (inability of tissue to extract oxygen) cyanide has high affinity for Ferric Iron (Fe3+), which inhibits cytochrome C oxidase in the mito administer inhaled amyl nitrate to oxidize the ferrous iron (Fe2+) to ferric iron (Fe3+) , which generates an induced methemoglobin methemoglobin can't carry O₂ but has has high affinity for Cyanide; cyanide binds the Fe3+ rather than the mitochondrial cytochrome enzymes, diminishing the toxic effect

Answer 49

AKA hereditary nonpolyposis colorectal cancer HNPCC DNA mismatch repair mutation (MSH2 mutation) progress to colorectal cancer proximal colon ALWAYS involved ``` CEOS: colorectal endometrial ovarian skin ```

Answer 50

INR is used for following patients on Warfarin - calculated from PT - Warfarin is a Vit K antagonist (2,7,9,10 factors) - decreases in these factors, esp Fact 7, prolongs PT in the extrinsic pathway activated PTT is used to monitor unfractionated heparin, which primarily affects the intrinsic coagulation pathway (Hep = PTT)

Answer 51

commonly used to prevent ischemic stroke in pts w/ TIA it irreversibly inhibits COX enzymes at low doses, it predominately inhibits COX-1 (preventing plt synthesis of Thromboxane-A2, which impairs plt aggregation and reduces vasoconstriction) this leads to risk of GI bleed: - inhibition of plt aggregation - impairment of prostaglandin-dependent GI mucosal protection PPIs can help protect GI bleed risks in Aspirin pts

Answer 52

C > A > B Flecainide flakes is clutching his jar of PB while he eats in bed next to his Propafenone The Quinidine queen and procainamide king casually eat their PB the Lidocaine lady and mexiletine Mexican are too concerned about lying to pay attention to their PB

Answer 53

drugs that end in -rubicin form free radicals in myocardium; cumulative dose-related dilated cardiomyopathy presents w/ L and R ventricular CHF dilated cardiomyopathy via swelling of SR, then loss of cardiomyocytes (myofibrillar dropout)

Answer 54

Vit B12 deficiency- destruction of gastric mucosa in body/fundus, destroying parietal cells (which normally secrete intrinsic factor and HCl) presents w/ megaloblastic anemia, LE paresthesias, and fatigue parietal cells found in upper glandular layer of stomach, just deep to columnar epithelial cells no parietal cells means no IF, which is required for B12 absorption in jejunum and duodenum

Answer 55

responsible for gallbladder contraction, increasing pancreatic enzyme secretion (via acinar cells), and decrease gastric emptying produced by I cells in duodenum and jejunum in response to fatty acts and AAs

Answer 56

cervical = head/neck hilar = lungs mediastinal = trachea/esophagus axillary - upper limb, breast, skin above umbilicus celiac = liver, stomach, spleen, pancreas, upper duodenum Superior mesenteric = lower duodenum, jejunum, ileum, colon to splenic flexure inferior mesenteric = splenic flexure to upper rectum internal iliac = lower rectum to anal canal (above pectinate line), bladder, vagina (middle third), cervix, prostate para-aortic = testes, ovaries, kidneys, uterus superficial inguinal = anal canal (below pectinate line), skin below umbilicus (except popliteal), scrotum, vulva popliteal = dorsolateral foot, posterior calf

Answer 57

SAD PUCKER ``` Suprarenal (adrenal) glands Aorta and IVC Duodenum (2,3,4th) Pancreas (except tail) Ureters Colon (descending and ascending, NOT transverse) Kidneys Esophagus (thoracic) Rectum (partial) ```

Answer 58

splenic flexure and retrosigmoid junction ]they lie between regions of perfusion of major arteries they're particularly susceptible to ischemic damage 2/2 hypotension

Answer 59

PPI amoxicillin clarithromycin (macrolide)

Answer 60

MSH2- Lynch Syndrome/HNPCC APC- FAP, sporadic colon cancer VHL- VHL p53- Li-Fraumeni syndrome MEN1- MEN1 RET- MEN2

Answer 61

Adenocarcinoma: Barrett esophagus (metaplasia to intestinal-type columnar epithelium cells) GERD SCC: tobacco alcohol achalasia (dysmotility)

Answer 62

Scleroderma: triad of autoimmunity, vasculopathy, and collagen deposition Diffuse scleroderma: widespread skin involvement, rapid progression anti-Scl-70 Ab ``` Limited scleroderma: limited skin involvement (fingertips and face) CREST syndrome Calcinosis (around veins) Raynaud phenomenon Esophageal dysmotility Sclerodactyly (thick skin) Telangiectasia Anti-centromere Antibody! ```

Answer 63

synthetic analog of somatostatin inhibits the release of vasodilator hormones, thus causing splanchnic vasoconstriction, which decreases portal flow, and decreases vatical bleeding also treats acromegaly also treats diarrhea in various endocrine disorders

Answer 64

chief cells: in gastric body and antrum secrete pepsinogen ``` D cells: gastric antrum and duodenum secrete somatostatin (inhibitory effects), which suppresses gastrin, insulin, CCK, pancreas secretions, and bile flow ``` G cells: gastric antrum secrete gastrin, which stimulates secretion of gastric acid by parietal cells and pepsinogen by chief cells Parietal cells: gastric mucosa secrete HCl S cells: Small intestine secrete secretin in response to low duodenal pH to protect SI mucosa from acid and increase bicarb secretion from pancreas and bile ducts I cells: duodenum and jejunum secrete CCK in response to FAs and AAs

Answer 65

MMCs are migrating motor complexes they occur every 90-120 min between meals, beginning in stomach and going to ileum, then die out general cleansing function, sweeping undigested food, debris, and bacteria through intestine to prep for next meal eating disrupts MMC in absence of MMCs, you'll get bacterial overgrowth Motilin produces the MMCs during fasting states Motilin is only found in small intestine

Answer 66

Hashimoto Thyroiditis

Answer 67

HLA-DR3 and HLA-DR4 Imagine: Type "1", (not type 2, so skip 2), then 3 and 4

Answer 68

serum IgA = mucosal immunity

Answer 69

Sabin = live attenuated IgA mucosal response IgG response this vaccine gives you potential to infect others via stool shedding, so it's not used in US Salk = inactivated/killed IgG only; used in United States

Answer 70

posterior duodenal wall = gastroduodenal artery; picture duodenum traveling directly in front of stomach Lesser curve of stomach = R gastric artery (or L gastric off celiac) Greater curve of stomach = L gastroepiploic artery (off of gastroduodenal) duodenum to proximal 2/3 of transverse colon = SMA

Answer 71

GI gut hormones called Incretins stimulate pancreatic insulin secretion in response to sugar-containing meals Incretin secretion is independent of blood glucose levels (happens before rise in blood glucose, too) Incretin activates GLP-1 and GIP IV glucose increases insulin 2/2 sensitivity of pancreatic beta cells' response to blood glucose

Answer 72

fructose metabolism occurs in liver essential fructosuria: asymptomatic defect in fructokinase fructose can't go to F1P, glycerol, or glycolysis, etc. but fructose is not trapped inside cells because it can go back to glucose via Hexokinase (you have a problem w/ fructose metabolism, but it's "kind enough" not to interfere w/ your life) fructose intolerance: symptomatic defect in aldolase B, which takes F1P--> DHAP or glyceraldehyde you have hypoglycemia, jaundice and vomiting after fructose ingestion (fruit, juice, honey) urine dipstick is neg (only looks at glucose) tx: diet- don't eat fructose OR sucrose (glu + fructose)

Answer 73

Very Poor Carb Metabolism Type 1- Von Gierke Type 2- Pompe Type 3- Cori Type 5- McArdle all of them cause accumulation of glycogen inside of cells, therefore PAS stain positive! these are all auto recessive (there's 12 total)

Answer 74

Type 1 glycogen storage disease Defect in Glucose 6 Phosphatase, which normally converse G6P back to glucose Impaired gluconeogenesis and glycogenolysis (cannot go "backwards" up in the last step of the pathway chart) ``` Presents: SEVERE fasting hypoglycemia very high glycogen in liver high blood lactate high triglycerides high uric acid (Gout) hepatomegaly ``` Tx: frequent oral glucose Avoid fructose and galactose

Answer 75

Type 2 glycogen storage disease Defect in alpha-1,4-glucosidase AKA acid maltase takes place in lysosomes only it's supposed to remove the entire glucose-chain nub off the molec Presents: Cardiomegaly, hypotonic muscles in infant "Pompe trashes the pumps" incl heart, liver, and muscles, leading to early death <2yo pts do not have hypoglycemia problems Treat: give alpha-1,4-glucosidase

Answer 76

Type 3 glycogen storage disease Defect in debranching enzyme alpha-1,6-glucosidase (the "b" in debraching looks like a 6 maybe?) it takes the final glucose nub off the branched molecule- imagine the b bump being the last nub Presents: milder form of Von Gierke gluconeogeneis is intact, but glycogenolysis isn't normal blood lactate levels accumulation of " limit dextrin like" structures in cytosol

Answer 77

Type 5 glycogen storage disease defect in glycogen phosphorylase in skeletal muscles (myophosphorylase) which is supposed to take the big glucose chains off the molec Presents: high glycogen in muscles, but muscle cannot break it down painful muscle cramps myoglobinuria w/ exercise arrhythmias from electrolyte abnormalities "Second-wind" phenomenon due to increased muscular blood flow blood glucose levels are typically unaffected "Mcardle = Muscle"

Answer 78

``` fatty acid synthesis: "Citrate = Synthesis" in well-fed state start with citrate in mitochondria use the citrate shuttle to go to cytoplasm then need ATP citrate lyase to turn into Acetyl-CoA then go to Malonyl-CoA then go to FA synthesis (palmitate) ``` fatty acid degradation: "Carnitine = killing/carnage of fatty acids" in a starved state: start w/ Fatty acid + CoA in cytoplasm use Fatty acid CoA synthase to go to Fatty acyl-CoA then use carnitine shuttle to go to mito matrix then undergo beta-oxidation w/ Acyl CoA dehydrogenase to make Acetyl CoA Acetyl CoA can turn into ketone bodies or go to the TCA cycle **Malonyl CoA inhibits the transfer of Fatty acid to the mito matrix via inhibiting carnitine acetyltransferase; therefore inhibiting FA degradation/beta oxidation

Answer 79

high cholesterol or bilirubin, low bile salts, and gallbladder stasis all lead to stones cholesterol stones: most common obesity, Crohn, age, estrogen therapy, multiparty, rapid weight loss, Native Americans Pigment stones: Ca + unconjugated bilirubin black- Calcium bilirubinate, hemolysis brown- infection; releasing beta-glucuronidase by injured hepatocytes and bacteria, which increases unconjugated Bilirubin seen w/ Crohn, chronic hemolysis, alcoholic cirrhosis, age, biliary infections, TPN (bile stasis)

Answer 80

defect in a middle step in turning glucose into the TCA cycle defect in Pyruvate Dehydrogenase Pyruvate cannot go to Acetyl CoA, which is supposed to go to TCA cycle (transition point from glycolysis to TCA cycle) build up of pyruvate, which gets shunted to lactate (via LDH) --> lactic acidosis and also shunted to alanine (via Alanine aminotransferase ALT) Findings: neuro defects, lactic acidosis, high serum alanine starts in infancy treatment: ketogenic diet- high in Lysine and Leucine ("Lyle and Lucy" eat keto); they cannot be metabolized to pyruvate and will not lead to increased production of Lactic Acid --low carb/glucose diet so you don't have to do glycolysis

Answer 81

defect in Lactate Dehydrogenase LDH normally helps in converting pyruvate to Lactate in the Cori Cycle it converts NADH to NAD+ when O₂ is depleted (exercising muscle), you use LDH for anaerobic glycolysis if you have LDH deficiency, then you have low NAD+, which leads to fast muscle fatigue/breakdown

Answer 82

decrease GH and TSH used to treat gastronoma, acromegaly, carcinoid syndrome, glucagonoma, and esophageal varices somatostatin comes from pancreatic delta cells overproduction causes decrease in secretin, CCK, glucagon, insulin, and gastrin

Answer 83

``` 4 major causes of hypoxemia, which is low PaO2 (low O₂ in arteries): alveolar hypoventilation V/Q mismatch diffusion impairment R-->L shunting ``` pAO2 is normally 104mmHg, and by the time it becomes systemic, it drops to 100 (bronchial circulation blood mixes), so an Aa gradient is normally between 5-15mmHg. If a pt is hypoxemic with a normal Aa gradient, then you know the low PaO2 is directly due to the low pAO2; so it's not due to V/Q mismatch or an O₂ diffusion impairment. Hypoxemia with a nl Aa gradient can be caused by: alveolar hypoventilation or high altitude

Answer 84

Hypertrophic cardiomyopathy this is a sarcomere protein defect autosomal dominant

Answer 85

HCC | germ cell tumors

Answer 86

CA 19-9 = pancreatic CA 125 - ovarian

Answer 87

GI (colorectal)

Answer 88

choriocarcinoma | germ cell tumors

Answer 89

patchy infiltrates = bronchopneumonia alveolar walls w/ inflammatory infiltrate = interstitial entire lobe = lobar 4 lobar stages: congestion (first 24 hrs) macro: affected lobe is red, heavy, and boggy micro: vascular dilation; and alveolar exudate contains mostly bacteria Red hepatization (2-3 days) macro: red, firm lobe ("liver-like") micro: alveolar exudate contains erythrocytes, neutrophils, and fibrin Gray hepatization (days 4-6) macro: gray-brown firm lobe micro: RBCs disintegrate; alveolar exudate contains neutrophils and fibrin Resolution Macro: restoration of nl architecture micro: enzymatic digestion of exudate

Answer 90

obstructive: WOB is minimized by breathing slow, deep breaths (lower rate and high TV) Restrictive: WOB is minimized by breathing fast, shallow breaths (higher rate and low TV) higher TVs increase the work needed to counteract the elastic resistance of the lung

Answer 91

Cystic Fibrosis: recurrent pulm infections, chronic bronchitis/bronchiectasis --> reticulonodular CXR pancreatic insufficiency w/ malabsorption, steatorrhea, and fat-soluble vitamin deficiency Infertility in men (absent Vas Deferens; spermatogenesis may be normal), subfertility/thick cervical mucus in females Nasal polyps, nail clubbing Kartagener syndrome (primary ciliary dyskinesia): immotile cilia 2/2 defective dynein M/F infertility (immotile sperm and dysfunctional fallopian tube) risk of ectopic pregnancy bronchiectasis, recurrent sinusitis situs inversus (dextrocardia CXR)

Answer 92

``` MEN 1: PPP Parathyroid (hypercalcemia) Pituitary (PRL, visual) Pancreatic (esp gastrinoma) ``` ``` MEN 2A: PPM Parathyroid Pheochromocytoma (adrenal) Medullary Thyroid Cx (Calcitonin) ``` ``` MEN 2B: PMM Pheochromocytoma Medullary Thyroid Cx Mucosal neuromas/Marfanoid Habitus ```

Answer 93

immediately: minimal/no change up to 1 Day: coagulative necrosis, edema, hemorrhage, wavy fibers, marginal contraction bands up to 1 Week: coag necrosis w/ neutrophil, then macrophage infiltrate 1 Month: continued macrophage phagocytosis granulation tissue w/ neovascularization collagen deposition/scar formation

Answer 94

mediates paraneoplastic cachexia in humans via suppressing appetite (hypothalamus) and increasing basal metabolic rate

Answer 95

Auer rods, which stain with myeloperoxidase Pt will present with anemia, leukopenia, and thrombocytopenia symptoms as a result from the marrow being replaced by leukemic cells- often shows up as DIC t(15:17) gives APL subtype, which is associated with PML/RARalpha fusion gene the APL subtype responds to all-trans-retinoid acid ATRA- Vitamin A

Answer 96

KRAS is a porto-oncogene that encodes a GTP-associated protein (GAP) associated with Epidermal Growth Factor Receptor EGFR, among others, which promotes increased cell proliferations and growth Active Ras is bound to GTP and uses GAP to inactivate it with GDP. Ras mutations cause it to be permanently activated, which activate the MAP kinase pathway and activate transcription. KRAS mutations are found in different types of cancers, including a large portion of metastatic colon cancers

Answer 97

identifying unabsorbed fat and confirming malabsorption from a stool sample useful in initial eval of a pt with signs of malabsorption

Answer 98

``` pre-T lymphoblasts express CD2 CD3 CD4 CD5 CD7 CD8 ``` pre-B lymphoblasts express CD10 CD19 CD20 TdT is seen in both

Answer 99

common in children T-cell ALL can present as a mediastinal mass, that causes mass-effect such as SVC syndrome or tracheal compression w/ dyspnea and stridor, or esophagus w/ dysphagia B-cell ALL is more common but doesn't present w/ mass effect- it presents w/ fever, malaise, bleeding, bone pain, HSM

Answer 100

Reed Sternberg Cells seen on a background of lymphocytic infiltrates

Answer 101

Area postrema in 4th ventricle is AKA chemoreceptor trigger zone for vomiting The Nucleus Tractus Solitarius (NTS) is in the medulla. It receives info from the area postrema, GI tract via Vaugs nerve, vestibular sys, and CNS 5 major receptors contribute to the vomiting reflex: M1 muscarinic (motorcycle in the parking lot) D2 dopaminergic (2 D athlete ropes) H1 histaminic (sailors swatting bees) 5-HT3 serotonergic (shot put balls) Neurokinin 1 (NK1) (plaNK1 horse) 5-HT3 and NK1 receptor antagonists are particularly useful for chemotherapy-induced vomiting. 5-HT3 blocker is ondansetron (happy dancer in front of shot put) NK1 blocker is Substance P and Aprepitant (urine test from participants)

Answer 102

most common childhood brain tumor, usually in cerebellum GFAP positive Rosenthal fibers (eosinophilic corkscrew fibers) Imagine a child laying w/ his hair on a pillow looking up at stars and roses, and considering FAPping lolz

Answer 103

childhood brain tumor located exclusively in cerebellum highly malignant- "drop metastases" into cauda equina primitive neuroectodermal tumor can compress 4th ventricle--> noncommunicating hydrocephalus see many small blue cells that form Homer Wright rosettes (groups of cells surrounding the neuropil) imagine Adam Sandler in the Waterboy w/ "medulla" scene- he's not communicating well, while the teacher has the big head (hydrocephalus). He knows his momma at Home is Wright, and he drops off 4 rosettes on his horse

Answer 104

childhood brain tumor ependymal tumors most commonly found in 4th ventricle can cause hydrocephalus characteristic perivascular rosettes rod-shaped blepharoplasty (basal ciliary bodies) found near nucleus Imagine 4 roses, but someone has drawn on vasculature w/ a pen

Answer 105

childhood brain tumor may be confused with pituitary adenoma (both cause bitemporal hemianopsia) derived from remnants of Rathke pouch- anterior pituitary Calcification is common cholesterol crystals found in "motor-oil" like fluid within tumor- "wet keratin" Imagine a pharyngioma Pharaoh forcing you to experience his Rathke by pushing you into a PIT of bones (calcium) and eat carrots dipped in motor oil and cholesterol

Answer 106

childhood brain tumor pineal gland tumor can cause Parinaud syndrome (compression of tectum --> vertical gauze palsy) obstructive hydrocephalus precocious puberty in M (beta-hCG production) histologically similar to germ cell tumors Imagine looking up (vertical gaze) and being paranoid (Parinaud syndrome) of God (vertical) getting the big head (hydrocephalus) and obstructing (obstructive) all pine trees (pineal)

Answer 107

AKA grade IV astrocytoma common, highly malignant in cerebral hemispheres; crossing corpus callosum = butterfly glioma "pseudopalisading" pleomorphic tumor cells - border central areas of necrosis and hemorrhage GFAP positive stain Imagine butterflies gliding (glio) across all hemispheres of the earth, almost as if they're para-sailing (pseudo-palisading), but they're actually FlAPping their wings. They border the palace areas w/ necrosis and hemorrhage

Answer 108

common, typically benign brain tumor most often near surfaces/falcine and parasagittal regions arise from arachnoid cells, extra-axial, and may have dural attachment (tail) often asymptomatic, but may get HA worsening w/ sleep see spindle cells in a whorled pattern and psammoma bodies Imagine dreaming (sleep) that an MAN (meningioma) arachnoid spider w/ a tail (dural attachment) on your bathroom shower porcelain surface (falcine/parasagittal surface). He can go unnoticed (asymptomatic), but momma (psammoma) decides to turn the water on and watch him whirl(whorled spindle cells) around the drain anyway

Answer 109

associated with von Hippel-Lindau syndrome when found w/ retinal angiomas. can produce EPO, leading to secondary polycythemia close, thin-walled capillaries w/ minimal intervening parenchyma imagine an erythro- Hippo who's got bloodshot eyes staring at a He-Man named Lin

Answer 110

classically at cerebellopontine angle, but can be along peripheral nerves often localized to CN 8--> vestibular schwannoma. Bilateral vestibular schwannomas associated with NF-2 Schwann cell origin, S-100 positive presents w/ hearing loss/tinnitus, loss of balance histo: spindle cells w/ palisading nuclei arranged around Verocay bodies (eosinophilic cores) imagine 100 deaf swans wearing "velcro" vests who fall into BOTH SIDES (bilateral, NF-2) of the pink pond edge (eosinophils, cerebellopontine angle) because they lost their balance parasailing around

Answer 111

most often in frontal lobes, rare, slow growing "chicken wire" capillary pattern oligodendrocytes = fried eggs- round cells w/ clear cytoplasm often calcified Rarely, we-all-go get breakfast w/ chicken, fried eggs, and white calci-Yum milk

Answer 112

most commonly a prolactinoma, or nonfunctioning bitemporal hemianopsia from optic chiasm compression could also be lactotroph, gonadotroph, somatotroph, or corticotroph

Answer 113

pulsatile GNRH in hypothalamus goes to pituitary gland pituitary gland releases FSH and LH LH stimulates release of testosterone form Leydig cells of testis FSH stimulates the release of Inhibin B from Sertoli cells Testosterone has a neg feedback effect on LH and GnRH Inhibin B has a neg feedback effect on FSH FSH levels may be higher in M w/ only 1 testicle since they have fewer sertoli cells producing the inhibitory Inhibin B

Answer 114

commonly found in young F with pressure to be thin may be seen w/ F who used to have nl menses but they're now irregular/absent due to decreased circulating leptin levels 2/2 diminished adipose tissue stores. decreased leptin inhibits pulsatile GnRH lease, which decreases LH and FSH secretion, circulating estrogen, and amenorrhea

Answer 115

auto dominant LDL receptor malfunction leading to accelerated atherosclerosis and early-onset CAD 70% of plasma LDL is normally cleared by the liver's LDL receptors

Answer 116

Papillary carcinoma: most common; ionizing radiation (acne tx) is common risk factor papillae w/ "orphan annie eye" cells and nuclear grooves (coffee beans), and psammoma bodies Annie cells described as "large cells with finely dispersed chromatin and ground-glass appearance) Imagine "orphan annie" doesn't have a pappi or a psammoma Follicular carcinoma: follicle surrounded by a capsule WITH invasion "hope your cap doesn't break if you fall" Medullary carcinoma: associated with MEN2 and RET gene parafollicular C cells (neuroendocrine cells that secrete calcitonin) calcitonin may deposit as amyloid, which stains apple green with Congo Red "Amy tries to "C" a pair of 2 Men at the Congo, but Ed, Lary and Cal say "no no" Anaplastic carcinoma: undifferentiated malignant cells in elderly large pleomorphic giant cells invades local structures producing dysphagia or resp compromise (similar to Riedel Fibrosing Thyroiditis in young pt)

Answer 117

tumor of pancreatic alpha cells, causing overproduction of glucagon presents w/ hyperglycemia (often as newly diagnosed diabetes mellitus) and necrolytic migratory erythema (blistering erythematous plaques w/ central clearing around groin, face, extremities, may transform to bronze/brown central indurated area w/ peripheral blistering and scaling) ``` Presents w/ 5 D's: Dermatitis Diabetes DVT Declining weight Depression ```

Answer 118

``` DIC: patients bleed coag cascade is activated PT and PTT are prolonged low fibrinogen and increased FDP (fibrin degradation products, esp D-dimer) ``` ``` TTP-HUS: usually do not bleed only plts are activated normal PT/PTT normal Fibrinogen ```

Answer 119

vWF: normal or prolonged PTT prolonged bleeding pts often present w/ lifelong hx of mucosal bleeding nl platelet levels but prolonged bleeding due to impaired plt functioning Hemophilia A: high PTT, but normal bleeding time characteristic deep-tissue bleeding (into joints, GI bleeds, hematuria)

Answer 120

Squamous cell carcinoma: alcohol and tobacco consumption of N-nitroso-containing foods (smoked Japanese); also hot liquids Histology shows ovoid epithelial cells w/ eosinophilic cytoplasm, keratin pearls, and intercellular bridging; commonly with large hyerpchromatic cells w/ bizarre nuclei and atypical mitoses ``` Adenocarcinoma: Barrett's esophagus GERD Obesity Tobacco Adenocarcinoma is more common in America, so correlate that to Barrett's/GERD ```

Answer 121

VEGF | FGF

Answer 122

decreased serum haptoglobin (bind free Hb; decrease when large quantities of Hb are released into circulation) increased LDH increased bilirubin

Answer 123

it's an indolent B cell neoplasm bone marrow infiltration causes fibrosis, bone marrow failure, and pancytopenia, so you get a "dry tap" massive splenomegaly (crossing midline) from spleen red pulp infiltration histo shows lymphocytes w/ cytoplasmic projections

Answer 124

t(8;14) affects c-myc gene large jaw mass histo shows starry sky pattern

Answer 125

CML the mutation increases tyrosine kinase activity

Answer 126

follicular lymphoma t(14;18) translocation generalized LAD normally, BCL2 inhibits apoptosis

Answer 127

neuroblastoma

Answer 128

spotty necrosis with ballooning degeneration (hepatocyte swelling w/ wispy/clear cytoplasm), councilman bodies (eosinophilic apoptotic hepatocytes), and mononuclear cell infiltrates pts present acutely w/ fever, malaise, anorexia, N/V, RUQ pain several days later have cholestasis, jaundice, pruritus, dark-colored urine (increased conjugated bilirubin levels), and echoic stool (lacks bilirubin pigment) self-limited, doesn't progress to HCC

Answer 129

absence of GnRH neurons in hypothalamus 2/2 defective migration from olfactory placode pts have central hypogonadism and anosmia; may have other midline defects (cleft lip) often present w/ delayed puberty some public hair because adrenarche occurs normally

Answer 130

Rh: Rh(-) mom has an Rh(+) baby, some fetal blood enters maternal circulation (delivery), and mom creates anti-D IgG against Rh antigen. Rh(-) mom has an Rh(+) baby again, but this time has the IgG plasma antibodies, which crosses placenta and causes erythroblastosis fetalis. To prevent this, give the mother anit-D IgG (RhoGAM) during third trimester, which prevents maternal anti-D IgG production. Babies will have positive direct Coombs test (indicating autoimmune hemolysis), anemia, jaundice, and edema/hydrops fetalis, immature/nucleated RBCs, and extra medullary hematopoiesis (HSM). ABO: type O mother has an A,B, or AB fetus. She's had IgG (the non-pregnancy ex has IgM written?) antibodies against these her entire life.

Answer 131

Hb Barts = gamma tetramers (in utero) no alpha globin chains; causes hydrops fetalis HbF = alpha2gamma2 HbA = alpha2beta2 HbA2= alpha2delta2 HbC= alpha2betaLysine2 (mutated beta chain- "C has lysine") HbS= alpha2betaS2 (mutated beta)

Answer 132

vWF disease vWF normally binds GP1b on plts to allow for plt adherence, then aggregation. vWF also carriers Factor 8 and prolongs its half life. vWF deficiency impairs plt function (with nl plt numbers) and presents as easy bruising and mucocutaneous bleeding (gingival, menses). A functional deficiency of Factor 8 also leads to prolonged bleeding after minor procedures. Ristopetin activates platelet GP1b receptors and makes them available for vWF binding. but if vWF levels are decreased, there's poor plt aggregation in the presence of ristocetin.

Answer 133

glutamic acid --> Valine | ex. GAG --> GTG

Answer 134

mutation in HFE protein. HFE normally interacts w/ transferring receptor to form a complex that senses the status of iron stores. Inactivating HFE mutations causes RBCs and hepatocytes to detect falsely low iron levels, so iron accumulation is increased 2 ways: Enterocytes increase transport/absorption from GI lumen Hepatocytes decrease hepcidin synthesis, allowing for increased iron secretion in circulation and causing Fe overload Fe overload gives you triad: micro nodular cirrhosis diabetes mellitus skin pigmentation- "bronze diabetes" increased risk for HCC, CHF, and testicular atrophy

Answer 135

CD14 think TB also CD40, CCR5, MHC class 2, B7 (CD80/86), Fc and C3b receptors (enhanced phagocytosis)

Answer 136

CD16 (binds Fc of IgG) CD56 (unique for NK) don't require thymus to be made member of innate immunity induced to kill when exposed to nonspecific activation signals on target cells, or if there's an absence of MHC class 1 on target cell surface also kills via antibody-dependent cell-mediated cytotoxicity (CD16 binds Fc region of Ig, which activates NK cell)

Answer 137

CD34 these cells can go on the myeloid path, or the lymphoid path

Answer 138

CD8 | CXCR4/CCR5 (co-receptors for HIV)

Answer 139

CD4, CD40L

Answer 140

TCR (binds antigen-MHC complex) CD3 (associated with TCR for signal transduction) CD28 (binds B7 on APC) CXCR4/CCR5 (co-receptors for HIV)

Answer 141

A3 = hemochromatosis B8 = Addison disease, Myasthenia Gravis B27 = "PAIR" Psoriatic arthritis, Ankylosing spondylitis, IBD-associated arthritis, Reactive arthritis/Reiter AKA seronegative arthropathies DQ2/DQ8 = Celiac disease "I ate too much gluten at Dairy Queen" DR2 = MS, SLE, Goodpasture DR3 = DM1, SLE, Graves, Hashimoto, Addison DR4 = Rheumatoid arthritis, DM1, Addison "4 walls to a room" DR5 = Pernicious anemia --> Vit B12 deficiency, Hashimoto

Answer 142

B cells: recognize antigen, and undergo mutation to optimize antigen specificity produce antibody, and differentiate into plasma cells to secrete specific Immunoglobulins Maintain immunologic memory, and memory B cells persist and accelerate further response to antigen T cells: CD4+ T cells help B cells make Ab's and produce cytokines to recruit phagocytes and activate other leukocytes CD8+ T cells directly kill virus-infected cells participate in delayed cell-mediated hypersensitivity (Type 4) Participate in acute and chronic organ rejections

Answer 143

Hot T-bone stEAK ``` IL-1: fever (hot) IL-2: stimulates T cells IL-3: stimulates Bone marrow IL-4: stimulates IgE production IL-5: stimulates IgA production and eosinophils IL-6: stimulates acute-phase reactants ```

Answer 144

all nucleated cells (not mature RBCs)

Answer 145

IL-1: causes fever and acute inflammation induces chemokine secretion to recruit WBCs IL-6: production of acute-phase proteins IL-8: major chemotactic factor for neutrophils- PUS "clean up on aisle 8"- neutrophils recruited by IL-8 to clear infections IL-12: differentiation of T cells into Th1 cells activates NK cells TNF-alpha: mediates septic shock, WBC recruitment, vascular leak, causing fever, hypotension, leukocytosis, and tachycardia causes cachexia in malignancy!

Answer 146

all T cells: IL-2 and IL-3 Th1 cells: Interferon-gamma TH2 cells: IL-4, IL-5, IL-10 IL-2: stimulates T cells: stimulates growth of helper, cytotoxic, and regulatory T cells; and NK cells IL-3: stimulates BONE marrow supports growth/differentiation of bone marrow stem cells Interferon-gamma: from Th1 cells and NK cells secreted in response to IL-12 from macrophages; stimulates macrophages to kill phagocytosed pathogens; inhibits differentiation of Th2 cells; activates NK cells to kill virus-infected cells ``` IL-4: from Th2 cells induces differentiation of T --> Th2 cells promotes growth of B cells enhances class switching to IgE and IgG ``` IL-5: from Th2 cells promotes growth/differentiation of B cells enhances class switching to IgA stimulates growth/differentiation of eosinophils IL-10: from Th2 cells and Treg cells attenuates inflammatory response decreases expression, inhibits activation "TGF-beta and IL-10 both atTENuate the immune response"--- WOUND HEALING

Answer 147

part of innate host defense against viruses they are glycoproteins that are synthesized by virus-infected cells that act locally on uninfected cells, "priming them" for viral defense by helping to selectively degrade viral nucleic acid and protein "interfere w/ viruses'

Answer 148

Hemophilia you could give Factor 8 or 9, depending on if it's A or B/Christmas, or you could give Thrombin Activated Factor 10a goes to 5a, which turns prothrombin into thrombin (Factor 2a), which turns fibrinogen into fibrin (Factor 1a) then Factor 13a is used to make a cross-linked fibrin clot remember thrombeaver has 2 front teeth and fibrin/fibers are usually very thin/stick-like, so they're represented as a 1

Answer 149

T cells arrive in sub capsular zone of thymus: CD4 neg and CD8 neg "double negative" T cells migrate to the cortex, where they gain CD4 and CD8 markers "double positive" there's positive selection where only T cells expressing a TCR that is capable of binding self MHC are allowed to survive. otherwise, they're eliminated T cells migrate to the medulla, where they interact w/ medullary and dendritic cells and become "single positive" of either CD4+ or CD8+ negative selection occurs by eliminating cells when there's excessive affinity for self-antigens or MHC. this serves to eliminate T cells that may be overly reactive against self-antigens and may contribute to autoimmunity if not destroyed. the CD4+ cells go on to become helper T cells the CD8+ cells go on to become cytotoxic T cells

Answer 150

ALL not PC at all, but remember that Coach W had ALL......

Answer 151

Philadelphia chromosome, t(9:22) this fuses the BCR-ABL genes

Answer 152

yeast = circles hyphae = smooth branches pseudohyphae = branches w/ notches blebs at the end = candida

Answer 153

defect in heme synthesis 2/2 X-linked defect in delta-ALA synthase gene can be genetic, acquired, or reversible (alcohol, lead, Vit B6 deficiency, Cu deficiency, INH meds) Heme is made of Fe and protoporphyrin ``` to make Protoporphyrin, Succinyl CoA +ALAS + VitB6 --> ALA (+ALAD) --> porphyobilinogen --> protoporphyrin ``` then, in the mitochondria, protoporphyrin + Fe via ferrochelatase to make Heme if this process is disrupted, Fe is trapped in the mitochondria, Heme isn't made, and you get ringed sideroblasts

Answer 154

both result in an intrinsic, hemolytic, normocytic anemia (high Reticulocytes) G6PD deficiency: G6PD provides NADPH needed to reduce Glutathione in order to let it continue to neutralize H2O2 via oxidation G6PD deficiency means you're susceptible to oxidative stress; severity depends on how severe the half life of G6PD is decreased (African = mild; Mediterranean = severe) the oxidative stress puts Hb as Heinz bodies, which are removed in spleen to make bite cells Pyruvate kinase deficiency: has elevated indirect bilirubin you have low ATP, giving you rigid RBCs, causing extravascular hemolysis from spleen (splenomegaly), causing echinocyte "burr cell" w/ small uniform projections

Answer 155

Fab (Fragment, antigen binding) consists of light and heavy chains that recognize antigens. it's the top 2 branches of the Y Heavy chain is the majority of the Y, and the light chain is the 2 extra lines off the top of the Y The Fc region is the trunk of the Y (so Heavy chain contributes to Fc and Fab regions. Light chain only contributes to Fab.) Fc is constant, carboxyl terminal, complement binding, carb side chains, and determines isotope (IgM, IgD, etc)

Answer 156

mature, naive B cells express IgM and IgD prior to activation (you get to "be" an "MD" once you're mature, even if you're naive) B cells may differentiate into plasma cells by isotope switching. the plasma cells secrete IgA, IgG, or IgE (you differentiate as you AGE) IgG: main Ab in delayed response to antigen most abundant crosses placenta IgA: prevents attachment to mucus membranes in GI tract most produced, but lower serum concentrations released into secretions (tears, mucus, breast milk) picks up the secretory component from epithelial cells which protects the Fc portion from luminal proteases IgM: produced in the primary/immediate response to antigen does not cross placenta its pentamer form allow avid antigen binding while the humoral response evolves IgD: unclear function. surface of many B cells IgE: binds mast cells and basophils cross-links when exposed to allergen (asthma) mediates immediate hypersensitivity (type 1) via release of inflammatory mediators, such as histamine mediates worm immunity by activating eosinophils lowest concentration ins serum "IgG is relevant during Gestation" "IgA sees things you Ate" "IgM is an iMMediate responder" "IgE is allerg-E responsive and activates Eosinophils"

Answer 157

``` Right shift: ACE BATs right handed, and "unloads" (O₂) on the ball: Acid (high H+, low pH) CO₂ Exercise 2,3-BPG Altitude Temp there's decreased affinity of Hb for O₂ (facilitates unloading into tissue) ``` L shift: a decrease in the above factors (low H+, high pH) facilitates O₂ loading in the lungs (decreased unloading) Left = Lower peripheral tissues have higher concentrations of CO₂ and H+, which facilitate O₂ unloading from Hb (Bohr effect) lung tissue O₂ binding to Hb releases H+ and CO₂ into a low-concentration environment (Haldane effect)

Answer 158

production of a mutant Factor V making it resistant to degradation by protein C, causing a hyper coagulable state. Complications incl DVT, cerebral vein thrombosis, recurrent pregnancy losses

Answer 159

via alternative splicing- where exons of the gene are reconnected in multiple ways during post-transcriptional processing results in different mRNA sequences which gives you different proteins

Answer 160

AIP: defective PBG deaminase (porphobilinogen deaminase) you get build up of PBG and D-ALA in urine symptoms are the 5 P's: Painful abdomen Port wine-colored urine Polyneuropathy PSYCH DISTURBANCES (EARLY) Precipitated by drugs/alcohol, starvation Treatment: glucose and heme to inhibit ALASynthase Porphyria cutanea tarda: defect in uroporphyrinogen decarboxylase results in uroporphyrin in urine (tea-colored) Presents LATE WITH bolstering photosensitivity Lead poisoning: early disorder = neuropsych (defect in D-ALAD) late disorder = photosensitivity (defect in ferrochelatase)

Answer 161

Aplastic anemia: progressive signs/symptoms of pancytopenia thrombocytopenia- bleeding anemia- pallor/fatigue leuko/neutropenia- infections absent splenomegaly, since there's lack of available hematopoietic progenitor cells/ no extra medullary hematopoiesis bone marrow bx is hypo cellular Parvovirus B19: aplastic crisis, but red cell aplasia only (pancytopenia in immunocompromised pts) bone marrow bx is hypo cellular Myelodysplastic Syndrome: defect in stem cell maturation, leading to pancytopenia Bone marrow bx is hyper cellular with misshapen cells/nuclei

Answer 162

associated with HHV-8 and HIV endothelial malignancy most commonly of skin, but also mouth, GI, and resp tract. frequently mistaken for bacillary angiomatosis, but has lymphocytic infiltrate blue-violet or brownish skin plaques

Answer 163

5' capping via addition of 7-methylguanosine cape poly A tail addition via polyadenylation of 3' end intron splicing out polyadenylation signal = AAUAAA poly-A polymerase does not require a template cytoplasmic P bodies play a role in mRNA translation regulation and degradation in the cytoplasm-- mRNA's quality control

Answer 164

Growth hormone binding surface receptors cytokines (interferon) hematopoietic growth factors (EPO, G-CSF)

Answer 165

MUDPILES ``` methanol (formic acid) uremia DKA Propylene glycol Iron tablets or INH Lactic acidosis Ethylene glycol (antifreeze) Salicylates (aspirin) ``` use Winter's formulas to check the compensation for a simple metabolic acidosis

Answer 166

Predicted respiratory compensation for a simple metabolic acisosi can be calculated using the Winters formula. measured CO₂ = lab value predicted CO₂ = calculated CO₂ calculated pCO2 = 1.5 [HCO3-] + 8 +/- 2 lab > calc = superimposed resp acidosis lab < calc = superimposed resp alkalosis (appropriate compensatory response) a superimposed respiratory acidosis means your CO₂ is still high; you haven't blown off enough; respiratory failure should be considered

Answer 167

HARDASS ``` Hyperalimentation Addison's Renal tubular acidosis Diarrhea Acetazolamide Spironolactone Saline infusion ```

Answer 168

Hyperventilation is the cause ``` hysteria hypoxemia (high altitude) salicylates (early) tumor pulm embolism ```

Answer 169

hypoventilation is the cause ``` airway obstruction acute lung disease chronic lund disease opioids, sedatives weakening of resp muscles ```

Answer 170

loop diuretics vomiting (lose HCl) antacids hyperaldosteronism loop diuretics respond to saline; hypoaldosteronisim is not saline responsive ``` CLEVER PD contraction (dehydration) licorice/laxative Endocrine (Conn's) Vomiting/GI loss Excess alkali (antacids) Renal (Bartter's), severe hypokalemia Posthypercapnia Diuretics ```

Answer 171

Thiamine is Vit B1 think ATP: alpha-ketoglutarate dehydrogenase (TCA cycle) Transketolase (HMP shunt) Pyruvate dehydrogenase (links glycolysis to TCA cycle) Branched-chain ketoacid dehydrogenase (maple syrup urine disease)

Answer 172

3 D's of B3: Pellagra Dermatitis (sun-exposed areas- broad collar rash) Dementia Diarrhea Hartnup disease: auto receive disease of neutral AA (tryptophan) transporters in renal tubules and RBCs; leads to low tryptophan for conversation into Niacin treat with high protein diet and nicotinic acid

Answer 173

insulin release by pancreatic beta cells is stimulated by increased ATP production Glukokinase functions as a glucose sensor in pancreatic beta cells by controlling the rate of glucose entry into the glycolytic pathway mutations in glucokinase cause maturity onset diabetes of the young (worsens during pregnancy)

Answer 174

benign liver and brain tumor "bloody sponge" or "mulberry cluster" of vasculature appearance w/ dilated vessels separated by thin CT septa biopsy is contraindicated due to risk of hemorrhage in the brain, it could cause focal/neuro deficits

Answer 175

entrapped median nerve, causing decreased sensation of first 3 fingers and thenar atrophy (but not loss of sensation there) median nerve courses between humeral and ulnar heads of pronator teres then between flexor digitorum superficialis and flexor digitorum profundus you could relieve symptoms by cutting the transverse carpal ligament (AKA flexor retinaculum) in the carpal tunnel associated with dialysis-related amyloidosis, pregnancy, Rheumatoid arthritis, hypothyroidism, DM

Answer 176

compression of ulnar nerve at wrist, classically in a cyclist 2/2 handlebar pressure

Answer 177

movement disorder sudden, wild flailing of 1 arm +/- ipsilateral leg caused by damage to contralateral sub thalamic nucleus (part of basal ganglia), which normally plays a role in inhibition of the thalamus most commonly due to lacunar stroke (2/2 longstanding HTN and DM)

Answer 178

Huntington caudate nucleus is part of Basal ganglia

Answer 179

Wilson disease specifically the putamen, because the lentiform nucleus made of putamen and globus pallidus

Answer 180

S1,2- buckle my shoe L3,4- kick the door C5,6- pick up sticks C7,8- lay them straight L1,2- testicles move (cremaster reflex) S3,4- winks galore (anal wink reflex)

Answer 181

pt is locked in their own body, often quadriplegic except for their eyes caused by rapid correction of hyponatremia rapid correction of hypernatremia will cause cerebral edema/herniation- commonly of Basilar artery "low to high, the pons will die" "high to low, your brain will blow"

Answer 182

LR6(SO4)3 CN 6- lateral rectus (abduct eye) -Abducens Damage presents as a medially directed eye that cannot abduct CN 4- Superior oblique (up and in) -trochlear Damage presents as upward eye movement, esp w/ contralateral gaze; pt will often oil head toward the side of the lesion (compensatory head tilt in opposite direction) pts will have trouble going down stairs and may complain of vertical diplopia CN3- the rest -oculomotor Damage presents as ptosis, "down and out" gaze if the motor output is damaged; diminished/absent pupillary light reflex, blown pupil if the parasympathetic output is damaged. -Commonly seen in vascular diseases, incl DM and sorbitol accumulation

Answer 183

located in tegmentum portion of brainstem (Between ventral and dorsal) Lateral nuclei = aLar plate (sensory) --sulcus limitans -- Medial nuclei = Motor (basal plate) ``` Nuclei: 4 above Pons 4 in Pons 4 below Pons CN 1,2 Midbrain- CN 3,4 Pons- CN 5,6,7,8 Medulla- CN 9,10,12 Spinal cord- CN 11 ```

Answer 184

imagine the red, yellow, and blue diagram on p. 478 Anterior cranial fossa: has cribriform plate, and CN1 Middle cranial fossa (through sphenoid bovine), has optic canal (CN2) Superior orbital fissure (CN 3,4,5-1, 6) foramen Rotundum (5-2) foramen Ovale (5-3) foramen spinosum- Middle meningeal artery ``` Posterior cranial fossa (through temporal or occipital bone) has Internal auditory meatus (CN 7,8) Jugular foramen (CN 9,10,11) Hypoglossal canal (CN 12) Foramen magnum (brainstem) ``` Remember for CN5: SRO Remember "9, 10, 11 = jugular foramen"

Answer 185

AKA lateral medullary syndrome AKA Wallenberg syndrome posterior inferior cerebellar artery (PICA) occlusion; can occur w/ cervical spine trauma w/ dissection of vertebral artery causes vomiting, vertigo, nystagmus, decreased pain/temp sensation from ipsilateral face and contralateral body; dysphagia, horseless, ipsilateral Horner syndrome, ataxia, dysmetria "don't PICA stag horn (nystagmus, horner's) horse (hoarseness) that can't eat (dysphagia) or follow a wall (ipsilateral; Wallenberg)"

Answer 186

middle cerebral artery stroke: contralateral paralysis and sensory loss- face and upper limb can cause Wernicke's aphasia if in temporal lobe can cause Broca's aphasia if in frontal lobe Aphasia if in dominant (~left) hemisphere Hemineglect if in non-dominant (~right) hemisphere also causes "pie" vision problems Anterior cerebral artery stroke: contralateral paralysis and sensory loss- LOWER limb

Answer 187

a common location of lacunar infarcts 2/2 unmanaged HTN the hypertensive vasculopathy involves the penetrating branches of the major cerebral arteries. the most frequently affected location is basal ganglia (putamen). the basal ganglia are supplied by lenticulostriate arteries, which are small branches off the MCA small lake-like infarcts in striatum that accumulate and create one significant hemorrhage

Answer 188

ipsilateral tongue dysfunction (tongue deviates ipsilaterally)

Answer 189

contralateral hemianopia with macular sparing!

Answer 190

synaptophysin = neurons GFAP = glial origin astrocytomas, ependymomas, oligodendrogliomas

Answer 191

thalamic nuclei VPL: receives input from spinothalamic tract and dorsal columns (drawn in your diagrams) -pain, temp, pressure, tough, vibration, proprioception VPM: receives input from trigeminal pathway (Makeup goes on the face) -face sensation they both send somatosensory projections to the cortex via thalamocortical fibers damage to both results in complete contralateral sensory loss Lateral geniculate nucleus: receives "L"ight input from CN2 Medial geniculate nucleus: receives "M"usic input from superior olive and inferior colliculus of tectum

Answer 192

mesocortical: decreased activity = negative symptoms (antipsychotic drugs have limited effect) mesolimbic: increased activity = positive symptoms primary therapeutic target of antipsychotic drugs (to decrease the positive symptoms) Nigrostriatal: major dopaminergic pathway in brain decreased activity = extrapyramidal symptoms (controls coordination and voluntary movement; significantly affected by movement disorders and drugs) tuberoinfundibulnar: decreased activity = increased prolactin and associated effects

Answer 193

INO: commonly seen in MS damage to MLF in dorsal pons caused by reduced saltatory conduction with relative preservation of axons eye on ipsilateral side of lesion has trouble adducting when looking in the opposite direction (if the lesion is on the left and you try to look right- the left eye cannot look towards your nose) convergence and pupillary light reflex are preserved

Answer 194

Some Lovers Try Positions That They Can't Handle ``` Scaphoid Lunate Triquetrum Pisiform --> Trapezium Trapezoid Capitate Hamate ``` start closest to thumb on both lines

Answer 195

Scaphoid- most commonly fractured (from a FOOSH) prone to avascular necrosis (retrograde radial artery blood supply) Lunate dislocation- may cause carpal tunnel syndrome Hook of hamate- from FOOSH can damage ulnar nerve

Answer 196

an upper humerus injury: fractured surgical head of humerus or anterior dislocation of humerus presents w/ flat deltoid loss of abduction at shoulder loss of sensation over deltoid and lateral arm

Answer 197

upper trunk compression presents: loss of elbow flexion loss of sensation over lateral forearm

Answer 198

mid shaft humerus fracture, passing through supinator canal, compression of axilla (crutches), or sleeping over chair (saturday night palsy) presents: wrist drop (loss of extension) decreased grip strength loss of sensation over posterior arm, forearm, and dorsal hand

Answer 199

supracondylar fracture of humerus (proximal lesion) carpal tunnel syndrome wrist laceration (distal lesion) presents: ape hand pope's blessing loss of wrist flexion loss of sensation over thenar eminence and parts of lateral 3.5 fingers Tinel sign (tingling on percussion) = carpal tunnel ``` recurrent branch: superficial palm laceration -presents: ape hand loss of thenar muscles but no loss of sensation ```

Answer 200

fracture of funny bone of humerus (medial epicondyle) or fracture of hook of hamate presents: ulnar claw on digit extension radial deviation of wrist loss of wrist flexion, adduction, flexion of 4th/5th digits loss of sensation over medial 1.5 fingers, incl hypothenar eminence

Answer 201

microglial nodules w/ subcortical dementia involves inflammatory activation of microglial cells, which form the nodules around small areas of necrosis

Answer 202

ALS causes both UMN and LMN lesions LMN lesion: loss of anterior horns in spinal cord causes muscle weakness and atrophy UMN: demyelination of lateral corticospinal tract causes spasticity and hyperreflexia mild atrophy of pre central gyrus gene mutation in superoxide dismutatse (SOD1) no bowel/bladder deficits treat with Riluzole, which decreases glutamate release

Answer 203

myotonic dystrophy, autosomal dominant caused by increased trinucleotide repeats difficulty releasing grip from a doorknob, ex My tonia, my testicles, my toupee, my ticker (myotonia, testicular atrophy, frontal balding, arrhythmia)

Answer 204

in "subacute combined degeneration" = Spinocerebellar tract lateral Corticospinal tracts Dorsal columns "back + 1 and 2" on each side = B12 presents w/ anemia, ataxia, paresthesias, impaired position/vibration, and positive Romberg

Answer 205

changes in the axon are called axonal reaction they include increased protein synthesis that facilitates axonal repair this is seen as enlarged, rounded cells with peripherally located nuclei and dispersed finely granular Nissl substance concurrent with Wallerian degeneration- degeneration of axon distal to site of injury (macrophages remove debris and myelin)

Answer 206

Dandy Walker: absent cerebellar vermis and cystic dilation of 4th ventricle; posterior fossa enlargement pts often present in infancy w/ skull enlargement and developmental delay associated with spina bifida Arnold Chiari malformation II: downward displacement of cerebellar vermis below foramen magnum pts frequently have spinal myelomeningocele non-communicating hydrocephalus from cerebral aqueductal stenosis

Answer 207

MG: auto-Ab's against postsynaptic ACh receptor- decreases endplate potential worsens with use associated with: thymoma Lambert Eaton: auto-Ab's against presynaptic Ca channels that decrease ACh release improves with use associated with small cell lung cancer

Answer 208

coagulative: seen in ischemia/infarcts except brain cell tissue architecture is preserved; anucleated cells liquefactive: bacterial abscess, brain infarcts neutrophils release "L"ysosomal enzymes to digest tissue (viscous liquid mass, pus, abscess, CSF-filled spaces) ``` Caseous: TB, fungi macrophages wall off --> granuloma cheesy, tan-white appearance "cottage cheese" ``` Fat: enzymatic: acute pancreatitis!! (saponification of fat) or traumatic (breast) damaged cells release lipase saponification of fat- chalky white appearance Fibrinoid: vessel wall damage, turning them thick and pink Gangrenous: distal extremity after chronic ischemia dry: coagulative wet: liquefactive superimposed

Answer 209

obturator: L2-L4 2/2 pelvic surgery (bladder cancer) causes decreased medial thigh sensation and decreased adduction Femoral: L2-L4 2/2 pelvic fracture, or a block in femoral crease (femoral nerve is not in femoral sheath) causes decreased thigh flexion and leg extension decreased sensation of anterior thigh loss of patellar reflex

Answer 210

both split from sciatic nerve (L4-S3), which innervates posterior thigh PED TIP Peroneal Everts and Dorsiflexes if injured, foot droPPED loss of sensation on dorsal foot -compensate w/ foot slaps Tibial Inverts and Plantarflexes if injured, you can's stand on your TIPtoes cannot curl toes or feel the sole of your foot

Answer 211

superior gluteal: L4-S1 upper medial gluteal region (if you inject something, go with upper lateral region; the lower region contains sciatic nerve) causes Trendelenburg sign- lesion is contralateral to the side that drops; the patient will lean toward the affected side to compensate for the hip drop (gluteus medius lurch) Inferior gluteal: L5-S2 injured in posterior hip dislocation causes difficulty climbing stairs or rising from seated position; loss of hip extension

Answer 212

lateral: hunger mediator. Stimulated by Ghrelin, inhibited by Leptin destruction leads to anorexia, failure to thrive. "if you zap your lateral area, you'll shrink laterally" ``` ventromedial: mediates satiety stimulated by leptin. damage causes hyperphagia "if you zap your ventromedial area, you'll grow ventrally and medially) ``` Anterior hypothalamus: controls cooling, parasympathetic (vasodilation, sweating). Damage causes hyperthermia A/C = anterior cooling Posterior hypothalamus: controls hearing, sympathetic. you vasoconstrict to prevent heat loss. "you get fired up. if you zap your posterior hypothalamus, you become a poikilotherm (coldblooded snake) and cause hypothermia Suprachiasmatic nucleus: controls circadian rhythm you need sleep to be charismatic

Answer 213

communicating: decreased CSF absorption by arachnoid granulations all ventricles are symmetrically enlarged Normal pressure hydrocephalus: a form of communicating hydrocephalus. Especially common in elderly, CSF pressure is only episodically elevated; no increased subarachnoid space volume. the corona radiata and the descending cortical fibers (bladder control) is distorted 2/2 expansion of ventricles. Classic triad of "wet, wobbly, wacky" with "magnetic gait" Noncommunicating hydrocephalus: caused by structural blockage of CSF within ventricles, so there will be enlargement above the obstruction and normal size below Ex vacuo: appearance of increased CSF on imaging, but it's actually 2/2 brain tissue loss (Alzheimers, HIV, Pick) ICP is normal, and no WWW triad is seen

Answer 214

sensory distribution past central sulcus in a coronal section ``` medial to lateral/inferior: distal toe--> leg --> up the trunk proximal arm --> hand forehead--> chin tongue --> swallowing ```

Answer 215

``` motor: CN 12 (hypoglossal) except palatoglossus muscle, which is CN 10 ``` general sensory: anterior 2/3 = mandibular branch of trigeminal posterior 1/3 = glossopharyngeal posterior area of tongue root: Vagus Gustatory (taste buds): Anterior = chords tympani branch of facial nerve posterior = glossopharyngeal tongue root, larynx, esophagus taste buds = Vagus

Answer 216

ipsilateral dysdiadochokinesia, limb dysmetria, intention tremor cerebellar hemispheres are responsible for planning motor and coordination of the ipsilateral extremities via connections w/ lateral descending motor systems

Answer 217

Haemophilus influenzae's virulence factor is cpolyribosylribitol phosphate, which is the capsule component of type b Hib.

Answer 218

virulence factor of Staph aureus found in the peptidoglycan cell wall binds Fc portion of IgG, impairing complement activation, opsonization, and phagocytosis

Answer 219

intensely pruritic rash, esp on flexor surfaces of wrist, lateral fingers, and finger webs delayed type 4 hypersensitivity rxn pts usually have excoriations w/ small, crusted, red papule scattered around dx confirmed with skin scrapings from excoriated lesions that show mites, ova, and feces

Answer 220

single stranded positive sense RNA in general, only RNA molecules purified from SS(+) viruses are infectious purified SS(-) RNA molecules and dsRNA molecules are not infectious, as they require additional enzymes (like an RNA polymerase)

Answer 221

Toxoplasma (cysts or tachyzoites) Cryptococcus (thick capsule yeast) Nocardia (GP rods)

Answer 222

intact cell-mediated immunity

Answer 223

can lead to increased CO₂ retention (oxygen-induced hypercapnia), which results in confusion and depressed consciousness. it's due to reversal of hypoxic pulmonary vasoconstriction, which increases physiologic dead space because the poorly ventilated alveoli are being perfused (V/Q mismatch)

Answer 224

mutation where Factor 5a is resistant to inactivation by activated protein C, leading to a hypercoaguable state. suspect this in a young pt with repeated unexplained DVTs and their plasma's PTT doesn't change when they're given Protein C

Answer 225

low CO₂ will cause increased cerebrovascular resistance, decreasing the blood volume and ICP in the brain

Answer 226

directly inferior/lateral to the lateral ventricles then, it's the internal capsule then, it's the Lentiform nucleus, made of the broad Putamen (broad men) and the narrow Globus palladus, making up the candy corn atrophied in Huntington

Answer 227

uncontrolled proliferation of all myeloid cells: RBCs, WBCs, and platelets it's a mutation in JAK2, a non-receptor (cytoplasmic) tyrosine kinase associated with the erythropoietin receptor

Answer 228

Giant cell (temporal) arteritis: headache, jaw claudication may lead to blindness associated with branches of the carotid artery, and polymyalgia rheumatica focal granulomatous inflammation with elevated ESR elevation CRP elevation IL-6! to mediate inflammation/acute phase reactants lesions are segmental, so you need a long bx section Takayasu arteritis: usually young Asian Females "pulseless disease" with weak UE pulses granulomatous thickening and narrowing of aortic arch and branch points

Answer 229

Polyarteritis nodosa: young adults lungs are spared classically involves renal arteries, mesenteric artery, and skin lesions Hepatitis B common transmural inflammation with fibrinoid necrosis; eventually fibroses and creates string of pearls Tx: cyclophosphamide and steroids ``` Kawasaki: Asian children <4yo rash on palms and soles coronary artery aneurysm, MI Tx: Aspirin and IVIG; disease is self-limited "child sitting on motorcycle w/ palms and soles w/ a racing heart" CRASH and burn: Chinese Conjunctival injection and Coronary Artery aneurysm Rash Adenopathy Strawberry tongue Hand-foot edema burn/fever ``` Buerger: necrotizing involving the fingers ulceration, gangrene, and autoamputation of fingers/toes Raynaud common Heavy smokers!!! (hypersensitivity to tobacco?) Tx: smoking cessation

Answer 230

Wegener Granulomatosis: AKA Granulomatosis w/ polyangiitis think of "C" disease: involves "C" shape of body, incl nasopharynx, lungs, and kidney (RPGN) c-ANCA Cyclophosphamide --nasal septum perforation, hematuria, lung granulomas Microscopic polyangiitis : similar to Wegener's, but NO nasopharyngeal involvement, so it still has lung and kidney involvement p-ANCA Tx: cyclophosphamide Churg-Strauss: AKA Eosinophilic Granulomatosis w/ polyangiitis granulomas w/ eosinophils, esp in lungs and heart Asthma, sinusitis, skin nodules p-ANCA (anti-neutrophil myeloperoxidase) Henoch-Schonlein Purpura (HSP): Vasculitis 2/2 IgA immune complex deposition; most common vasculitis in children PALPABLE PURPURA on buttocks and legs, GI pain, hematuria usually follows URI (IgA deposits drive the vasculitis) associated with IgA nephropathy (Berger disease)

Answer 231

First look at ACTH levels: if ACTH is suppressed, then you're either taking exogenous glucocorticoids or you have an adrenal tumor. both produce negative feedback to pituitary to decrease ACTH. if ACTH is elevated, then you know this is where the problem is coming from, and you need to do a dexamethasone test, a glucocorticoid. Low dose dex: it should normally kick the pituitary away and realize it should suppress ACTH (and therefore suppress cortisol) High dose dex: if it suppresses the ACTH/cortisol production, then you have a pituitary adenoma. If there is no suppression (ACTH remains high), then you have "ectopic ACTH production" which means it's coming from a malignant tumor that is resistant to negative feedback. Immediately think SMALL CELL LUNG CANCER

Answer 232

rare malignant neoplasms from mesothelial cells, which line body cavities (pleural, peritoneal, pericardial) asbestos is a primary risk factor (although it's more likely to cause lung cancer) Hemorrhagic pleural effusions and pleural thickening are seen histology shows long slender microvilli and abundant tonofilaments, and psammoma bodies serology markers: cytokeratin positive calretinin positive

Answer 233

chromaffin cells create a pheochromocytoma, causing excess production of catecholamines. mutated genes: VHL gene (Von Hippel Lindau) RET (MEN 2's) NF1 (neurofibromatosis) use the rule of 10s for these: 10% bilateral 10% extra-adrenal 10% malignant, etc

Answer 234

mutation in CFTR gene CFTR protein is a transmembrane ATP-gated chloride channel the CFTR is supposed to hydrate mucosal surfaces in airway and bowel, defects cause a misfolded protein, retained in the RER, and not transported to the cell membrane. this decreases Cl and water secretion, and also Na retention. this all causes thick, plugging mucus and elevated Na and chloride levels in sweat, and pancreatic damage clinically you see recurrent pulmonary infections, bronchitis, bronchiectasis, and reticulonodular CXR You get pancreatic insufficiency with pancreatic duct distension and obstruction, causing malabsorption, steatorrhea, biliary cirrhosis, liver disease, and infant meconium ileus. you can tx this w/ pancreatic lipase you also see infertility in M (absence of vas deferens), sub fertility in F (thick cervical mucus), nasal polyps!!, and nail clubbing

Answer 235

CD4+ cell-mediated destruction of parietal cells. Their ability to secrete HCl decreases, causing an elevated intraluminal pH, which upregulates gastrin secretion from G cells. The decreased parietal cells also means decreased intrinsic factor production, which means you get a Vit B12 deficiency. This manifests as megaloblastic anemia and subacute combined degeneration of spinal cord.

Answer 236

ethanol inhibits gluconeogenesis and can cause hypoglycemia once hepatic glycogen stores are depleted ethanol metabolism reduces NAD+ to NADH, which inhibits the pathways requiring NAD+, which include rxns for gluconeogenesis. Lactate cannot be converted to pyruvate, so instead the reaction is driven from pyruvate to lactate (lactic acidosis). excess NADH also inhibits the conversation of malate to oxaloactetate, which is an intermediate w/ pyruvate for gluconeogenesis

Answer 237

Hemosiderin-laden macrophages they come from pulmonary congestion, causing small, congested capillaries to burst/hemorrhage and leave Iron, which is then phagocytosed by macrophages stain blue with Prussian Blue stain

Answer 238

repetitive splenic infarctions caused by splenic micro vessel occlusion cause a shrunken, fibrosed, brown (hemosiderosis) spleen that eventually leads to autosplenectomy

Answer 239

``` Bell's Palsy: CN 7 palsy Motor: facial muscles Parasympathetic: lacrimal, submandibular/sublingual glands Special: taste anterior 2/3 tongue Somatic: afferents from ear ``` Horner Syndrome: sympathetic denervation of face, causing: Ptosis Anhidrosis Miosis (Pam is horny) Associated with lesion of spinal cord above T1

Answer 240

Degnerative/osteoarthritis: positional relieved w/ rest Radiculopathy/herniation: radiates to leg positive straight leg test Spinal stenosis: pain with standing relieved with spine flexion Spondyloarthropathy: Young M, HLA-B27 relieved w/ exercise prolonged morning stiffness spinal metastasis: constant worse at night not responsive to positional change vertebral osteomyelitis: focal tenderness fevers, night sweats recent infection, IV drug use, or immunocompromised

Answer 241

epidural: MMA rupture (high pressure) between skull and dura CT shows biconvex hematoma that does not cross suture lines presents w/ lucid interval after a severe head trauma subdural: bridging vein rupture between dura and arachnoid CT shows crescent shape that crosses suture lines presents as gradual onset of HA and confusion, often in atrophied brain (elderly/alcoholism) "banana, bridging veins, baby shakes" subarachnoid: (Berry) aneursym/AV malformation of ACA/PCA rupture (circle of willis) between arachnoid and pia CT shows blood at the base of brain; CSF is xanthochromic presents w/ "worse HA ever", nuchal rigidity; vasospasm can occur causing an eschmic infarct Intraparenchymal hemorrhage: AKA basal ganglia AKA putaminal hemorrhage: most commonly caused by systemic HTN, amyloid angiopathy (recurrent stroke), vasculitis. may be 2/2 reperfusion injury. Most commonly Basal Ganglia/ Charcot-Bouchard aneurysm of the lenticulostriate vessels

Answer 242

reversible bronchoconstriction, mostly due to allergies Curschmann spirals (shed epithelium forms whorled mucus plugs) Charcot-Leyden crystals (needle crystals with eosinophils)

Answer 243

can be a complication after acute pancreatitis collection of fluid rich in enzymes and inflammatory debris with a granulation/fibrosis wall it is not a true cyst, so it's not lined by epithelium

Answer 244

Laneway lesions are a vascular phenomenon: nontender lesions on palms and soles from micro emboli and splinter hemorrhages Osler nodes are an immunologic phenomenon: painful nodules seen on fingers and toes from immune complex deposition Roth spots are retinal hemorrhages with pale centers also an immunologic phenomenon all can be seen in bacterial endocarditis

Answer 245

Asbestos: affects lower lobes pleural thickening, "Ivory white" calcified pleural plaques are pathognomonic; asbestos (ferruginous) bodies are golden-brown fusiform rods resembling dumbbells w/ Prussian blue stain associated w shipbuilding, roofing, plumbing Beryllium: affects upper lobes noncaseating granulomas aerospace, manufacturing industries Coal Workers: affects upper lobes macrophages w/ carbon- inflammation and fibrosis AKA black lung disease Anthracosis: asymptomatic 2/2 urban dwellers exposed to sooty air Silicosis: affects upper lobes foundries, sandblasting, mines. Macrophages release fibrogenic factors and cause fibrosis. may increase susceptibility to TB. Birefringent silica particles "eggshell calcifications" on rim of hilar nodes "Asbestos is from the roof, but affects the base. Silica/coal are from the base, but affect the roof" except berylliosis, all contribute to risk of cor pulmonate, cancer, and Caplan Syndrome (RA and pneumoconiosis w/ intrapulmonary nodules)

Answer 246

Vitamin E deficiency: hemolytic anemia, muscle weakness, poster column and spinocerebellar tract demyelination Fredreich ataxia: an auto recessive degenerative condition has the ataxia from spinocerebellar degeneration, loss of position/vibration from dorsal column, and deep tendon reflexes from peripheral nerve demyelination B12 deficiency: similar neuro symptoms, but this has megaloblastic anemia, hyperhsegmented neutrophils, and increased serum methylmalonic acid levels

Answer 247

``` night blindness! severe eye dryness and corneal ulceration hyperkeratosis growth retardation dry, scaly skin Bitot spots on conjunctiva risk for squamous metaplasia ``` associated with PBC 2/2 malabsorption from biliary obstruction. so might also have pruritis 2/2 bile acid accumulation in skin

Answer 248

NER: specific endonucleases release the oligonucleotides w/ the damage DNA polymerase fills the gap ligase reseals the gap this repairs bulky helix-distorting lesions occurs in G1 phase defective in Xeroderma Pigmentosum (UV exposure damages pyrimidine dimers) ``` BER: base specific glycosylase removes the altered base and creates AP site Nulceotide(s) removed by AP-endonuclease, which cleaves 5' end lyase cleaves 3' end DNA polymerase-beta fills the gap DNA ligase seals it occurs throughout cell cycle important in spontaneous/toxic repair ``` ``` Mismatch repair: new strand's mismatched nucleotides are removed gap is filled and resealed occurs predominantly in G2 defective in Lynch Syndrome/HNPCC ```

Answer 249

F2,6BP activates phosphofructokinase-1, which increases glycolysis F2,6BP inhibits fructose 1,6- bisphosphatase, which decreases gluconeogenesis phsopfructokinase-2 increases F2,6BP in response to insulin fructose 2,6-bisphosphatase decreases F2,6BP in response to glucagon

Answer 250

Iron bound to heme is normally Fe2+, but nitrites cause poisoning by oxidizing iron to Fe3+, creating methemoglobin. Methemoglobin is unable to bind to oxygen, but the partial pressure of O₂ in the blood is unchanged. the partial pressure of O₂ represents the amount of O₂ dissolved in the plasma, and is unrelated to hemoglobin function. this means pO2 is also normal in CO poisoning and anemia. Methemoglobinemia's cause dusky skin (similar to cyanosis) and a functional anemia (unable to carry O₂)

Answer 251

it's responsible for intracellular effects of the Gs protein, where Gs activates adenylate cyclase adenylate cyclase converts ATP to cAMP cAMP activates protein Kinase A Protein Kinase A is used in several hormone receptors including TSH, glucagon, and PTH receptors=

Answer 252

receptor is activated, the alpha subunit undergoes conformational change, and that releases GDP and binds GTP. the GTP binding then allows the alpha subunit to dissociate from the remainder of the G protein complex to carry out its function

Answer 253

cGMP activation

Answer 254

JAK is a cytoplasmic protein activated by ligand binding to non-G protein-coupled transmembrane receptors that lack intrinsic tyrosine kinase activity. JAKs activate cytoplasmic transcription factors called STATs GH, EPO, and cytokines (like interferon) are common ligands for these receptors

Answer 255

ligands such as insulin and EGF act through transmembrane receptors that have intrinsic tyrosine kinase domains

Answer 256

cleaving cAMP to its inactive form, 5'-AMP

Answer 257

Fructose (fructose intolerance) but also sucrose, which has to be converted to fructose + glucose an aldolase B deficiency leads to a fructose-1-phosphate accumulation because it can't get converted to DHAP and glyceraldehyde. this is hereditary fructose intolerance. aldolase B deficiency impairs gluconeogenesis, so infants present w/ life-threatening hypoglycemia and vomiting after fructose or sucrose ingestion.

Answer 258

GALK deficiency: cannot convert galactose into galactose-1 phosphate in the normal lactose breakdown pathway (into galactose and glucose), so galactose gets shunted to galactitol via aldose reductase. this gives the patient galactosemia. patients have excess galactitol that causes cataracts and spills into urine. These may be the only 2 manifestations. Classic galactosemia comes from GALT deficiency, galactose-1-phosphate uridyltransferase. GALT deficiency is a more serious form of galactosemia that causes the galactitol accumulation in the lens, but it also has a toxic metabolite that causes hepatic and renal dysfunction. these pts present in neonatal period with vomiting, lethargy, and failure to thrive. treatment for these include excluding galactose and lactose (galactose + glucose) from diet switch breast milk to soy

Answer 259

hypothyroidism: high TSH (if primary) low free T3 and free T4 a total T3 may be normal hyperthyroidism: low TSH (if primary) high free or total T3 and T4

Answer 260

steroid production and phospholipid biosynthesis steroid producing cells are found in the adrenals, gonads, and liver. ACTH stimulates the steroid production in the adrenals

Answer 261

NAD and NADP Niacin is a precursor for NAD and NADH, which can be deficient in Alcoholics, causing pellagra

Answer 262

Serotonin syndrome: hyperreflexia and clonus NMS: hyporeflexia and rigidity

Answer 263

tryptophan

Answer 264

serotonin syndrome if it's combined with a serotonergic medication like SSRI, SNRI, or TCA because it has MAOI activity

Answer 265

Naltrexone- it's a mu antagonist. it blocks the rewarding and reinforcing side effects short term, you can use benzodiazepines to decrease withdrawals

Answer 266

sleep hygiene: maintain regular sleep/wake cycle and avoid naps avoid caffeine after lunch adjust bedroom environment to be cool, dark, and quiet exercise regularly but not before bed ``` stimulus control: use bedroom only for sleep and sex go to bed only when sleepy leave when unable to fall asleep fixed wake up time, including weekends ```

Answer 267

Medicare: >65 pts <65 with certain disabilities pts w/ ESRD Medicaid: joint federal and state for very low income pts medicarE for the Elderly medicaid for the Destitute Health Maintenance Organization: low monthly premiums low cost for pt strict guidelines (like a limited panel of providers) uses capitation: the payer pays a fixed, predetermined fee to provide all services. PPO: flexible, but expensive

Answer 268

increased appetite and sleep leaden paralysis rejection sensitivity mood reactivity MAOIs are good for this often treatment-resistant depression

Answer 269

Conduct disorder: C = childhood disorder repetitive and pervasive behavior violating the rights of others, including aggression to people and animals, destroying property, theft, etc. Turns into antisocial personality disorder Oppositional defiant disorder: Childhood pattern of hostile, defiant behavior towards authority figures in the absence of serious violations of social norms, like deliberately annoying peers. Antisocial personality disorder: A = Adult disorder disregard and violation of rights of others criminality, impulsivity. Usually has a history of Conduct disorder. "Antisocial = sociopath"

Answer 270

short: triazolam, oxazepam, midazolam intermediate: alprazolam, lorazepam, temazepam long: diazepam, chlordiazepoxide, flurazepam

Answer 271

``` Acetylcholine: made in basal nucleus of Meynert low in Alzheimer low in Huntington high in Parkinson "Save basal-cola at Meynards" ``` ``` Dopamine: made in ventral tegmentum, SNpc low in depression high in Schizophrenia high in Huntington low in Parkinson "the Substantial negro Teague men are very dope" ``` ``` GABA: made in nucleus accumbens (ventral striatum) low in Anxiety low in Huntington!!! "GABby comes to Bens for Hunting" ``` ``` Norepinephrine: Made in Locus ceruleus (rostral pons near lateral floor of 4th ventricle) high in anxiety low in Depression acts on dorsal pons for fight or flight "serious about NE-thing "pon" the floor" ``` ``` Serotonin: made in Raphe nucleus low in Anxiety low in depression high in Parkinson "No serotonin makes you so anxious you might Ralphe" ```

Answer 272

Hypocretin-1 (orexin-A) and Hypocretin-2 (orexin-B) are neuropeptides produced in the lateral hypothalamus that function to promote wakefulness and inhibit REM sleep-related phenomena.

Answer 273

AKA PCP hallucinogen that works primarily as an NMDA antagonist moderate doses cause dissociative symptoms. agitation, hallucinations, violent behavior can occur in high doses. Also, loss of coordination, horizontal and VERTICAL nystagmus, and cognitive symptoms including disorientation, poor judgement, and memory loss

Answer 274

facial droop from facial nerve compression. ``` the facial nerve exits skull through stylomastoid foramen and courses within parotid gland. then it divides into its 5 terminal branches: Temporal zygomatic buccal mandibular cervical "two zebras bit my cousin" ``` the trigeminal nerve's 3 branches are face sensation and mastication motor. and course deep into the face Horner syndrome is caused by interrupted sympathetic innervation to the head- lateral hypothalamus, hypothalmospinal tract, paravertebral sympathetic chain/ stellate ganglion, or internal carotid artery

Answer 275

junction of ipsilateral posterior communicating artery and internal carotid artery. the posterior communicating artery is the bottom R/L portions of the circle of willis, and CN3 wraps around this. it looks like it could also be anterior cerebral artery? CN3 problem will present as mydriasis with diplopia, ptosis, and "down and out" deviation of ipsilateral eye

Answer 276

CN 5- jaw deviates toward the lesion (unopposed force from opposite pterygoid muscle) CN10- uvula deviates away from lesion (weak side collapses and uvula points away) CN11- weakness turning the head to the Contralateral side of lesion (SCM). shoulder droops on the side of the lesion (trapezius). (the left SCM contracts to help turn the head to the right) CN12- LMN lesion. Tongue deviates toward the side of the lesion to "lick your wounds" (weakened tongue muscles on the affected side)

Answer 277

corneal: Afferent: 5-1 (nasociliary branch) Efferent: 7 (temporal branch: orbicularis oculi) lacrimation: A: 5-1 (loss of reflex does not preclude emotional tears) E: 7 jaw jerk: A: 5-3 (sensory- muscle spindle from masseter) E: 5-3 (motor- masseter) pupillary: A: 2 E: 3 gag: A: 9 E: 10 (cough: injury to internal laryngeal nerve, a branch off superior laryngeal nerve, off CN10)

Answer 278

cervical: wide white matter and wide grey matter H- squatty lateral dorsal column is thick anterior horn is thick thoracic: grey matter H is skinny both dorsal columns are present but skinny lumbar: thick grey matter H single dorsal column sacral: super thick grey matter H; almost takes up all the white cervical enlargement at C3-T1 lumbosacral enlargement at L1-S2

Answer 279

typically in fragile germinal matrix, common in prematurity shows blood in lateral ventricles

Answer 280

AKA waiter's tip traction or tear of upper (Erb-er) trunk: C5-C6 causes: lateral traction on infant neck during delivery trauma in adults deltoid/supraspinatus: can't abduct (arm hangs by side) infraspinatus: arm cannot laterally rotate (it's medial) biceps: arm cannot flex or supinate (it's extended and pronated) arm is hanging behind your back

Answer 281

traction or tear of lower trunk: C8-T1 causes: infants- upward force on arm during delivery Adults- trauma grabbing a tree branch intrinsic hand muscles are damaged (like Thoracic outlet syndrome) total claw hand: lumbricals normally flex MCP joints and extend DIP/PIP joints

Answer 282

compression of lower trunk and subclavian vessels causes: cervical rib pan coast tumor intrinsic hand muscles are damaged (like Klumpke) atrophy of intrinsic hand muscles; ischemia, pain, and edema due to vascular compression

Answer 283

lesion of long thoracic nerve causes: axillary lymph node dissection after mastectomy stab wounds chest tube placement (it traverses the serrates anterior) serratus anterior muscle is damaged causes inability to anchor the scapula to thoracic cage, so you cannot abductyour arm above the horizontal position. this is not the same as a shoulder droop

Answer 284

damage to spinal accessory CN11 nerve, in the posterior triangle damages the trapezius muscle causes you to have weak glenoid rotation upward

Answer 285

dominant: agraphia, acalculia, finger agnosia, left-right disorientation called Gerstmann syndrome non-dominant: hemispatial neglect syndrome (agnosia of the contralateral side of the world)

Answer 286

pineal gland mass classically causes obstructive hydrocephalus from aqueductal stenosis (papilledema, headache, and vomiting) germinomas are the most common pineal gland mass.

Answer 287

retina and optic nerve transmit the light stimulus to MIDBRAIN at the level of superior sulcus, where it's received by the pretectal nucleus and relayed to the bilateral Edinger-Westphal nuclei. These project parasympathetic fibers through the oculomotor nerve to the ciliary ganglion, which projects fibers to innervate the sphincter papillae muscle to constrict the pupil. when light is shown on the eye, both the ipsilateral pupil (direct response) and contralateral pupil (consensual response) constrict

Answer 288

sciatic nerve and piriformis piriformis syndrome occurs when the muscle is injured and compresses sciatic nerve, causing pain, tingling, and numbness of buttons and along the nerve distribution on posterior thigh

Answer 289

often due to inversion of a plantar-flexed foot most commonly involves the anterior talofibular ligament on the anterolateral aspect of the ankle

Answer 290

s= center spinal cord lesion loss of UE pain and temp loss of UE LMN signs and/or loss of LE UMN signs could be in setting of scoliosis a central cystic dilation in the cervical spinal cord slowly enlarges, which charastically causes damage to the ventral white composure and the anterior horns associated with Chiari 1 malformations where the cerebellar tonsils are pushed through foramen magnum

Answer 291

symmetric polyarthririts with prolonged morning stiffness and associated fatigue, improving with use (squeezing out inflammation). ``` Joint findings: subluxation, esp cervical spine ulnar deviated fingers swan neck fingers inflammatory synovial fluid! involves MCP, PIP, wrist NOT DIP or 1st CMC "soft, spongy, warm joints" ``` Many Extraarticular manifestations, incl: rheumatoid nodules in subQ tissue and lung, AA amyloidosis, Sjogren syndrome, etc. associated with: HLA-DR4 anti-CCP antibodies Rh+ (anti-IgG Ab) Tx: NSAIDs, glucocorticoids, disease-modifying meds

Answer 292

wear and tear- degenerative presents w/ pain in weight bearing joints, improving with rest asymmetric joint involvement no systemic symptoms ``` Joint findings: osteophytes (bone spurs) non-inflammatory synovial fluid INVOLVES THE DIP and PIP NOT MCP "hard, bony enlargements" ```

Answer 293

pts with CREST syndrome

Answer 294

autoantibodies targeting the Fc portion of human IgG the occur in most pts with RA poor specificity though- they're found in 10% healthy pts, 30% of pts with SLE, and nearly all pts with mixed cryoglobulinemia

Answer 295

``` >50 yo (usually F) with shoulder and hip girdle pain often with systemic symptoms muscle weakness is not common!! elevated ESR no cognitive impairment (like fibromyalgia) ``` also with giant cell arteritis with granulomatous inflammation of vascular media in temporal vessel rapidly responds to glucocorticoids

Answer 296

latissimus dorsi: innervated by the thoracodorsal nerve functions to extend, adduct, and internally rotate the humerus can be injured via forceful downward movement, incl throwing, climbing, or swinging a racquet overhead large muscle in lower back deltoid: innervated by axillary nerve abducts arm injured via sudden loading while in abduction infraspinatus: innervated by supra scapular nerve externally rotates arm injured with supraspinatus due to falls or overuse in older pts trapezius muscle: large upper back/neck muscle that elevates, rotates, and stabilizes the scapula innervated ~CN11 injured in whip-lash MVCs

Answer 297

SItS: Supraspinatus innervated by supra scapular nerve abducts arm initially (before deltoid takes over) most common rotator cuff injury- "empty can test" Infraspinatus supra scapular nerve laterally rotates arm pitching injury teres minor axillary nerve adducts and laterally (externally) rotates arm Subscapularis upper/lower sub scapular nerves medially rotates and adducts arm these are all primarily innervated by C5-C6

Answer 298

often involves CN3 caused by central nerve ischemia!, which affects somatic nerve fibers but spares peripheral parasympathetic fibers. this gives you ptosis, down and out gaze, and normal light/accomodation reflexes

Answer 299

``` both of them have: anti-Jo-1 + (anti-histidyl-tRNA synthetase) ANA + anti-Mi-2 antibody + anti-SRP + high CK ``` polymyositis: progressive proximal muscle weakness endomysial inflammation with CD8+ T cells most often involves shoulders ``` dermatomyositis: similar to polymyositis, but involves skin findings Malar rash Heliotrope (periorbital) rash "shawl and face" fresh "mechanic's hands" Gotton papules Perimysial inflammation and atrophy with CD4+ T cells ``` remember that perimysium is closer to the surface/dermis than the endomysium, so it has the derm findings

Answer 300

AKA uncal herniation compresses CN3 complication of an ipsilateral mass lesion, such as hemorrhage or brain tumor. the medial temporal lobe (uncut) herniates through the gap between the crus cerebri and the tentorium. first sign is a fixed and dilated pupil on the ipsilateral side, because the first thing you damage is preganglionic parasympathetic fibers running on the outside of CN3. Ipsilateral paralysis of the oculomotor muscles (down and out) may also see other things, like CL/IL hemiparesis, contralateral homonymous hemianopsia with macular sparing

Answer 301

rapidly progressive dementia with (startle) myoclonus spongiform transformation of grey matter Prions make beta pleated sheets which are resistant to proteases and accumulate

Answer 302

AKA Reactive arthritis can't pee, can't see, can't climb a tree conjunctivitis, urethritis, arthritis the arthritis is asymmetric, often involves a large joint, and frequently presents post-GI or chlamydia infections. but it gives you a sterile joint it's strongly associated with HLA-B27 but is a paiR spondyloarthropathy, so it's seronegative for Rheumatoid factor

Answer 303

part of the "pAir" seronegative (no rheumatoid factor) spondyloarthropties, with strong HLA-B27 association. it is symmetric involvement of spine and sacroiliac joints --> ankylosis (fusion), uveitis, and aortic regurg gives you bamboo spine, and you need to monitor breathing/chest wall expansion

Answer 304

AR mutation of ATP7B with excessive copper deposition into tissues 2/2 hepatic accumulation because its transport protein, ceruloplasmin, is dysfunctional: Liver (decreased secretion into biliary system) Basal ganglia cornea typically presents in young adults with liver disease, neuropsych symptoms (ataxia, personality, etc) and Kayser-Fleischer rings on slit-lamp diagnose with low Ceruloplasmin and high urinary Cu excretion, high Cu content on liver bx, and Kayser-Fleischer runs

Answer 305

tuberoinfundibulnar pathway it can be disrupted by D2 receptor blocking antipsychotics, so you could get hyperprolactinemia and galactorrhea and amenorrhea the mesolimbic and mesocortical pathways regulate condition and behavior the nigrostriatal pathway regulates coordination of voluntary movements

Answer 306

increased urate production: Primary gout- idiopathic myeloproliferative disorders (ex. polycythemia vera) or lymphoproliferative disorders tumor lysis syndrome HGPRT deficiency in the purine salvage pathway decreased urate clearance: chronic kidney disease thiazide or loop diuretic therapy

Answer 307

hypoxia = hippocampus - inability to form new memories histologic features of brain ischemia: 12-48hrs red neurons 24-72hrs necrosis + neutrophils 3-5 days macrophages (microglia) to phagocytose myelin breakdown 1-2 weeks: reactive gliosis and vascular proliferation >2 weeks: glial scar from astrocytes

Answer 308

AR trinucleotide repeat GAA disorder on chromosome 9 encoding frataxin (an iron-binding protein) impaired mitochondrial function degeneration of multiple spinal cord tracts, including spinocerebellar Friedreich is Fratastaic (frataxin): he's your favorite frat brother, always staggering and falling, but he has a sweet (DM) big heart (hypertrophic cardiomyopathy). kyphoscoliosis and foot abnormalities (pes cavus, hammer toes)

Answer 309

diaphragm, via the phrenic nerve roots passing through the interscalene sheath

Answer 310

pre patellar: inflammation of largest sac of synovial fluid can be caused by repeated trauma or pressure from excessive kneeling. it's directly anterior to patella supra patellar: caused from running superior to patella baker cyst: AKA popliteal cyst comes with chronic joint disease popliteal fluid collection in posterior popliteal fossa, commonly communicating with synovial space

Answer 311

Antinuclear antibodies anti-dsDNA antibodies anti-Smith antibodies antihistone antibodies (sensitive for drug-induced lupus) low C3, C4, and CH50 due to increased immune complex formation

Answer 312

rheumatoid arthritis

Answer 313

AMAs are associated with PBC

Answer 314

negative for rheumatoid factor, but HLA-B27 positive ankylosing spondylitis reactive arthritis IBD-associated arthritis psoriatic arthritis

Answer 315

achondroplasia: activating mutation in FGFR3 that inhibits chondrocyte proliferation, so it inhibits endochondral ossification short limbs, normal torso/head (preserved membranous ossification- this woven bone doesn't need cartilage) osteogenesis imperfecta ("type 1"): problem with bone matrix formation decreased production of otherwise normal type 1 collagen multiple fractures, blue sclera, hearing loss, tooth abnormalities (lack of dentin) Vitamin D deficiency: problem with bone mineralization

Answer 316

DMD is a mutation affection the dystrophin gene, which is a sarcolemma-cytoskeleton linker protein causes fibrofatty change, most notably in the calves

Answer 317

children: metaphysis of long bones, because of slower blood flow and capillary fenestrae in this region adult: vertebral body also the location of Pott disease (2/2 TB)

Answer 318

irreversible injury to a neuron characteristic changes become evident 12-24 hours after the event shrinkage of cell body eosinophilia of cytoplasm pyknosis of nucleus loss of Nissl substance

Answer 319

precision = reliability accuracy = validity

Answer 320

collects data from a group of people at a certain point in time. it measures exposure and outcome simultaneously (snapshot study). this includes measuring mean BP over the course of a week and comparing it to a protein mutation. looks at disease prevalence. can see risk factor association but does not establish causality

Answer 321

case control: ODD RATIO divides people into with disease vs without disease, then looks retrospectively for risk factors "think ok, you have COPD now. what are the ODDS that you were a smoker?" (saying what is the "risk" that you were a smoker doesn't make sense) OR = ad/bc cohort study: RELATIVE RISK divides people into risk vs no-risk, then looks to see if they have the disease can be prospective or retrospective "think ok, you smoke now, so you're at RISK for developing COPD RELATIVE to a non-smoker" RR = a/(a+b) / c/(c+d)

Answer 322

AR: the difference in risk between exposed individuals AR = [a/(a+b)] - [c/(c+b)] (20% risk of lung cancer in smokers vs 1% in non-smokers, AR = 20%) RRR: the proportion of the risk reduction that is attributable to the intervention compared to a control RRR = 1 - RR (2% pts w/ flu shot get the flu; 8% non vaccinated pts get the flu; RR = 2/8; RRR = 6/8) ARR: the difference in risk (not the proportion) attributable to the intervention compared to control ARR = [c/(c+d)] - [a/(a+b)] (8% of pts who receive placebo vaccine get the flu; 2% of pts who receive real vaccine get the flu; ARR = 8 - 2 = 6%

Answer 323

NNT = 1/ARR NNH = 1/AR

Answer 324

type 1 = alpha false positive error ("we're number 1 means you're falsely positive) type 2= beta false negative error beta is related to power, 1 - beta, which is the probability of rejecting the null hypothesis when it is truly false

Answer 325

CI = mean +/- 1.96 x (SD/(sqrtN)) standard error = SD/sqrtN

Answer 326

papillary cancer of the thyroid meningioma papillary serous carcinoma of the endometrium or ovary mesothelioma (pleura tumor)

Answer 327

stress: leakage with cough/sneeze caused by decreased urethral sphincter tone or urethral hyper mobility urge: sudden urge to urinate caused by detrusor hyperactivity overflow: incomplete emptying and persistent involuntary dribbling caused by impaired detrusor contractibility or a bladder outlet obstruction

Answer 328

via in vitro fertilization Turner syndrome most often causes infertility 2/2 ovarian failure. you could give sufficient estrogen and progestin to develop the thick endometrial lining to support a pregnancy.

Answer 329

noted 2-4 weeks after birth, a child will tilt its head to one side 2/2 malposition in utero or birthing trauma. resolves with conservative therapy and stretching

Answer 330

gastric tumor that's metastasized to ovary can present w/ weight loss, epigastric pain, and adnexal masses. histologically has large amounts of mucin and apically displaced nuclei, called signet ring cells

Answer 331

converts androgen to estradiol via FSH stimulation in the granulosa cells

Answer 332

``` Down Syndrome Trisomy 21 low alpha-fetoprotein **high beta-hCG low estriol **high Inhibin A ``` ``` Edwards Syndrome Trisomy 18 (election) (rocker bottom feet, clenched hands read to fight; low set ears, micrognathia) low alpha-fetoprotein low beta-hCG low estriol low/nl Inhibin A ``` ``` Patau Syndrome: Trisomy 13 (puberty) (rocker bottom feet, microcephaly, cleft lip/palate, holoprosencephaly, polydactyly) low beta-hCG **low PAPP-A (PAPP-A = Patau) ```

Answer 333

elevated FSH due to resistant ovarian follicles and lack of feedback from Inhibin.

Answer 334

``` Streak ovaries amenorrhea, infertility short stature webbed neck shield chest low posterior hairline bicuspid aortic valve neonatal lymphedema and cystic hygromaas ```

Answer 335

germline: mutation only in the egg or sperm cells the parents will be normal with a mutation in the offspring somatic: mutation from mitotic errors after fertilization mutations propagates through multiple tissues or organs of the patient

Answer 336

``` infundibulopelvic ligament (AKA suspensory ligament of the ovary): connects ovaries to lateral pelvic wall contains ovarian vessels you should ligate these vessels during oophorectomy to avoid bleeding you're at risk of ligating gonadal vessel when you ligate the ovarian vessels (it's fun to suspend the ovaries via vessels) ``` ``` cardinal ligament: connects cervix to side wall of pelvis contains uterine vessels ureter is at risk of injury during ligation of uterine vessels during hysterectomy ("cardinal connects cervix to side") ``` Round ligament: connects uterine fundus to labia major derivative of gubernaculum, traves through the round inguinal canal; above the artery of Sampson "Round Sampson is a major goober but he's still fun "IN ga canal" Broad ligament: connects uterus, fallopian tubes, and ovaries to pelvic side wall contains ovaries, fallopian tubes, and round ligaments of uterus it's the fold of peritoneum that comprises the mesosalpinx, mesometrium, and mesovarium "it's broad, so it contains everything" ovarian ligament: connects medial pole of ovary to lateral uterus derivative of gubernaculum; "ovarian Ligament Latches to Lateral uterus"

Answer 337

all part of broad ligament Mesosalpinx: fallopian tube to ovary Mesometrium: from the ovary down and lateral "it's the meat of the broad ligament" Mesovarium: blanket on top of the ovary keeps it "very warm"

Answer 338

suspensory ligaments (Cooper ligament) Paget disease of the nipple invades the lactiferous sinuses

Answer 339

acute mastitis: benign infection usually 2/2 Staph aureus associated w/ breast feeding purulent discharge periductal mastitis: inflamed subareolar ducts, usually 2/2 smoking Vit A deficiency causes squamous metaplasia, duct blockage, and inflammation classically presents as subareolar mass w/ nipple retraction 2/2 myofibroblasts causing granulating fibrosis ``` mammary duct ectasia: dilation of subareolar ducts postmenopausal multiparous F mass with green-brown nipple discharge (inflamm debris) plasma cells are characteristic ``` fat necrosis: trauma abnormal calcification (saponification) bx shows necrotic fat w/ calcifications and giant cells

Answer 340

``` fibrocystic change fibrosis and cysts in premenopausal F hormone mediated lumpy breast cysts have blue-dome appearance some are associated with increased risk for invasive carcinoma: apocrine metaplasia = no risk sclerosing adenosis = 2x risk atypical hyperplasia = 5x risk ``` intraductal papilloma fibrovascular projections w/ 2 cell layers bloody discharge in premenopausal F (vs papillary carcinoma in older F with 1 cell layer) fibroadenoma most common benign neoplasm, usually premenopausal F well circumscribed, mobile, marble-like mass Estrogen sensitive no risk of carcinoma ``` phyllodes tumor overgrowth of fibrous component "leaf like" projections postmenopausal F can be malignant "fibrous leaves fall in phyllodes" ```

Answer 341

DCIS: proliferation of ducts w/o BM invasion often see calcification, no mass Camedo-type has central necrosis and dystrophic calcification Paget disease has DCIS that extends to involve skin (this Paget's is almost always associated with underlying carcinoma) Invasive ductal Carcinoma: most common type; forms duct-like structures presents as mass or mammography may cause dimpling of skin or nipple retraction bx shows duct-like structures in a desmoplastic stroma 4 subtypes: Tubular: well-differentiated tubules w/ only 1 layer Mucinous: abundant mucin Medullary: high-grade cells w/ lymphocytes and plasma cells (BRCA1) Inflammatory: inflamed swollen breast w/ blockage of dermal lymphatics; may mistake for acute mastitis ``` LCIS: proliferation of lobules w/o BM invasion no mass/calcifications, often found incidentally dyscohesive cells w/o E-cadherin often multi-focal, bilateral Tx: Tamoxifen; low risk of progression ``` Invasive lobular carcinoma: single-file cells and no duct formation 2/2 no E-cadherin

Answer 342

increases maternal insulin resistance during 2nd and 3rd trimesters of pregnancy this leads to rise in serum glucose that helps provide adequate nutrition to growing fetus.

Answer 343

hypertensive disorder of pregnancy that gives you new-onset BP >20 weeks gestation AND proteinuria OR signs of end-organ damage

Answer 344

beta-hCG to make sure you don't have choriocarcinoma

Answer 345

for those who desire fertility, use clomiphene, an Estrogen Receptor modulator. it will decrease the negative feedback inhibition on the hypothalamus by circulating estrogen, thereby increasing gonadotropin production (FSH and LH), leading to ovulation for those who do not wish to become pregnant, give combined OCPs to minimize endometrial proliferation, reduce androgenic symptoms, and prevent unwanted pregnancy. Or, an androgen antagonist like spironolactone

Answer 346

1 week after conception in the serum and on home urine test 2 weeks after conception implantation occurs 6 days after fertilization (blastocyst sticks on day 6). the syncytiotrophoblasts secrete the hCG

Answer 347

septate uterus: incomplete resorption of the septum (in the last step), so you have a little v at the top of the uterus bicornate uterus: incomplete midline fusion of mullerian ducts, so you have a pretty wide V uterus didelphys: complete failure of fusion, so you have a double uterus, vagina, and cervix

Answer 348

internal iliac arteries

Answer 349

estrogen: increases HMG CoA reductase, which causes bile to become supersaturated with cholesterol progesterone: decreases bile acid production, and decreases gallbladder emptying

Answer 350

gap: made by connexin proteins for intercellular communication. useful in pregnancy tight: made by claudins and occludin to form paracellular barriers

Answer 351

adherens: via cadherins protein desmosomes: via cadherins protein (desmogleins, desmoplakin) hemidesmosomes: via integrins

Answer 352

46 xx, t(14;21)

Answer 353

DiGeorge syndrome presents with CATCH-22 Cleft palate Abnormal facies Thymic aplasia Cardiac defects (interrupted aortic arch, Tetralogy of Fallot) Hypocalcemia (2/2 parathyroid hypoplasia)

Answer 354

endometrial hyperplasia/carcinoma from chronic estrogen stimulation and decreased progesterone secretion (high LH:FSH ratio; the LH acts on the thecal cells to make androgens, which is converted to estradiol by FSH on granulosa cells and estrone by adipocyte cells)

Answer 355

perineal body | it's a tendinous center point of the perineum, and commonly cut during midline episiotomy

Answer 356

anaplastic cytotrophoblasts and syncytiotrophoblasts without villi often presents as dyspnea/hemoptysis 2/2 pulmonary metastasis from hematogenous spread

Answer 357

presents as large unilateral adnexal mass in a postmenopausal woman. high Inhibin levels high estrogen, causing endometrial hyperplasia (and precocious puberty in a young F) it's a sex cord stromal tumor, which has a predominance of granulosa cells and a scattering of theca cells. the theca cells are yellow with lipid. call-exner bodies can be seen (cells arranged in a micro follicular or rosette pattern)

Answer 358

iliohypogastric nerve often damaged in appendectomy

Answer 359

commonly from direct frontal trauma infraorbital nerve- paresthesia of upper cheek, upper lip, and upper gingiva. it's a branch off the maxillary nerve inferior rectus muscle- trouble with vertical gaze

Answer 360

transverse portion of duodenum is trapped between SMA and aorta, giving you partial intestinal obstruction this could occur when the artery branching angle decreases 2/2 decreased mesenteric fat, pronounced lordosis, or surgical correction of scoliosis

Answer 361

severe stenosis to one of the proximal subclavian arteries, causing the reversal of blooodflow from the contralateral vertebral artery to the ipsilateral vertebral artery. pts may have symptoms of arm ischemia, including exercise-induced fatigue, pain, and paresthesias, or vertebrobasilar insufficiency (dizziness, vertigo)

Answer 362

external abdominal aponeurosis and the transversalis fascia, respectively

Answer 363

commonly from meiotic nondisjunction during parental gametogenesis causing a 47XXY karyotype often with developmental delay and Barr body (inactivated X chromosome) dysgenesis of seminiferous tubules (sertoli cells) causes decreased Inhibin B, leading to high FSH the abnormal Leydig cells will cause low testosterone, high LH, and high estrogen ``` presents with: testicular atrophy euchnoid body shape tall, long extremities gynecomastia female hair distribution ```

Answer 364

X linked dominant inheritance- missing a tip of X Trinucleotide repeat (CGG) in FMR1 gene on X chromosome causing methylation, which means decreased expression. ``` presents with: intellectual disability Macroorchidism Long face, large jaw large everted ears autism mitral valve prolapse ``` Fragile X = extra large ears, jaw, testes

Answer 365

SRY gene on Y chromosome Sertoli cells re required for internal genitalia development. They secrete Inhibin B, which inhibits FSH. They secrete androgen binding protein ABP, which maintains high levels of LOCAL Testosterone, needed for internal genitalia development. They support sperm synthesis and regulate spermatogenesis. Temp sensitive. Convert T and androstenedione to estrogens via aromatase. No sertoli cells or lack of AMH (anti-Mullerian Hormone) gives pt both male and female internal genitalia and male external genitalia "testicles are tot-toil on the inside" Leydig cells: secrete T in the presence of LH. Unaffected by temperature. DHT is required for external genitalia and prostate "can't lie about your external genitalia"

Answer 366

inability to convert T to DHT you have ambiguous external M genitalia until puberty, when the increased T levels cause masculinization

Answer 367

Female: default development mesonephric duct degnerates; paramesonephric duct develops uses WNT-4, RSPO1, DAX-1, and FOX2-2 Males: Use the SRY gene on Yp11; produces TDF (testis-determining factor) Sertoli cells secrete AMH (MIF) that suppresses paramesonephric ducts. Leydig cells secrete androgens that stimulate mesonephric ducts. use SOX-9, FGF9 genes. High local T and AMH are required by the 8th week of development to develop the Mesonephric duct

Answer 368

AKA Mayer-Rokitansky Küster Hauser syndrome MRKH may present in Female with primary amenorrhea (lack of uterine development) but fully developed secondary sex characteristics (functional ovaries making normal estrogen)

Answer 369

having a tumor secreting hCG, which is similar to TSH. hCG is normally produced by placenta, but it's also produced in malignant testicular tumors, specifically nonseminomatous germ cell tumors.

Answer 370

testicles: para-aortic scrotum: superficial inguinal

Answer 371

structures supplied by the IMA: | left colic, sigmoid, and superior rectal arteries/structures

Answer 372

Finasteride: 5-alpha reductase inhibitor, decreasing T --> DHT used for BPH and male pattern baldness reduces prostate volume to increase urine flow Flutamide: competitive inhibitor at androgen receptors. Treats prostate carcinoma, which is dependent on androgen signaling for growth. Tamsulosin: alpha-1 antagonist specifically selective for receptors on prostate (vs vascular) treats BPH by inhibiting smooth muscle contraction

Answer 373

Ketoconazole: inhibits 17,2-desmolase-mediated steroid synthesis., preventing cholesterol conversion to androgens. it treats precocious puberty Spironolactone: K sparing; inhibits steroid binding, 17-alpha hydroxylase, and 17,20-desmolase it also blocks aldosterone binding, leading to Na/H2O loss both can be used to treat PCOS to reduce androgenic symptoms. both can cause gynecomastia and amenorrhea

Answer 374

cancers of the pelvis, including the prostate, like to spread hematogenously to the skeleton, specifically the lumbosacral spine via the vertebral venous plexus, which is connected to the prostatic venous plexus

Answer 375

posterior urethra injury presents with inability to void, full bladder sensation, high riding/boggy prostate, and blood in urethral meatus anterior urethra is most commonly damaged in straddle injuries, when the bulbous urethral segment and perineum is struck forcefully (crossbar of a bicycle) penile urethra is injured in penetrating trauma or instrumentation

Answer 376

hypospadias: incomplete fusion of urethral/urogenital folds. Females' urogenital folds do no fuse and ultimately form the labia minora. epispadias: abnormal positioning of genital tubercle in 5th week of gestation. it becomes penis in M and clitoris in F

Answer 377

hypospadias: incomplete fusion of urethral/urogenital folds. Females' urogenital folds do no fuse and ultimately form the labia minora. epispadias: abnormal positioning of genital tubercle in 5th week of gestation. it becomes penis in M and clitoris in F

Answer 378

high frequency tremor with sustained posture (outstretched arms, holding an object) that worsens with movement or anxiety often familial. pts often self-medicate with alcohol to decrease tremor amplitude. Tx with nonselective Beta blockers (propranolol)

Answer 379

sudden/stepwise decline in cognitive function with a series of ischemic strokes. glial scar formation on autopsy

Answer 380

they sense intracardiac filling pressures, so ANP and BNP are released to do 3 things: increase GFR via afferent vasodilation and efferent vasoconstriction decrease proximal Na+ reabsorption (promote Na excretion) decrease Renin secretion, which decreases Ang2 and Aldosterone levels all 3 of these functions promote natriuresis and diuresis to decrease the increased blood volume levels

Answer 381

Insulin receptor has 2alpha and 2 beta subunits the alpha subunits are extracellular, and provide binding site for insulin. the beta subunits are intracellular and obtain tyrosine kinase domains that are activated when insulin binds to alpha. a series of signaling is triggered, starting with autophosphorylation of insulin receptor (IR), phosphorylation of IR substrates 1 and 2 (IRS-1,2), and ultimately translocation of glucose transporter-4 (GLUT4) to the cell membranes via the PI3K pathway. Tyrosine phosphorylation of IRS activates the PI3K pathway to cause glycogen, lipid, and protein synthesis. It also activates the RAS/MAP kinase pathway, which causes cell growth and DNA synthesis. TNF-alpha is a pro-inflammatory cytokine that induces insulin resistance via activation of serine and threonine kinases. the Threonine and Serine kinases phosphorylate serine residues on the beta subunits of IR and IRS-1. This inhibits tyrosine phosphorylation of IRS-1 by IR and hinders downstream signaling, resulting in resistance to the normal actions of insulin. Catecholamines, glucocorticoids, and glucagon can induce insulin resistance in the same mechanism.

Answer 382

Insulin receptor has 2alpha and 2 beta subunits the alpha subunits are extracellular, and provide binding site for insulin. the beta subunits are intracellular and obtain tyrosine kinase domains that are activated when insulin binds to alpha. a series of signaling is triggered, starting with autophosphorylation of insulin receptor (IR), phosphorylation of IR substrates 1 and 2 (IRS-1,2), and ultimately translocation of glucose transporter-4 (GLUT4) to the cell membranes via the PI3K pathway. Tyrosine phosphorylation of IRS activates the PI3K pathway to cause glycogen, lipid, and protein synthesis. It also activates the RAS/MAP kinase pathway, which causes cell growth and DNA synthesis. TNF-alpha is a pro-inflammatory cytokine that induces insulin resistance via activation of serine and threonine kinases. the Threonine and Serine kinases phosphorylate serine residues on the beta subunits of IR and IRS-1. This inhibits tyrosine phosphorylation of IRS-1 by IR and hinders downstream signaling, resulting in resistance to the normal actions of insulin. Catecholamines, glucocorticoids, and glucagon can induce insulin resistance in the same mechanism.

Answer 383

Cerebral amyloid antipathy: lobar hemorrhage, esp elderly, tends to be recurrent; focal neuro deficits Charcot Bouchard aneurysm: chronic hypertension, hemorrhage involving the deep brain structures (basal ganglia, cerebellar nuclei, thalamus, pons) Hypertensive encephalopathy: progressive headache and N/V followed by non localizing neuro symptoms (confusion)

Answer 384

pancreatitis is a major risk factor! diffuse injury to pulmonary microvascular endothelium and alveolar epithelium, causing increased pulmonary capillary permeability and a leaky alveolocapillary membrane. this causes noncardiogenic pulmonary edema with a normal pulmonary capillary wedge pressure!!. Edema, inflammation, and hyaline membrane formation 2/2 ARDS decrease the lung compliance, increase work of breathing, and decrease the oxygen diffusion capacity in the lung with a worsening V/Q mismatch (an elevated PCWP would be more suggestive of cariogenic pulm edema, like decompensated LVF)

Answer 385

pancreatitis is a major risk factor! diffuse injury to pulmonary microvascular endothelium and alveolar epithelium, causing increased pulmonary capillary permeability and a leaky alveolocapillary membrane. this causes noncardiogenic pulmonary edema with a normal pulmonary capillary wedge pressure!!. Edema, inflammation, and hyaline membrane formation 2/2 ARDS decrease the lung compliance, increase work of breathing, and decrease the oxygen diffusion capacity in the lung with a worsening V/Q mismatch (an elevated PCWP would be more suggestive of cariogenic pulm edema, like decompensated LVF)

Answer 386

dilated and enlarged heart chambers- eccentric hypertrophy, with decreased ventricular contractility (systolic dysfunction) the heart is large and floppy. you get R and L heart failure signs, S3, systolic regurg murmur, and heart looks like a balloon on CXR. commonly after viral infection, familial, toxicity (alcoholism), pregnancy, and hemochromatosis it's the most common cardiomyopathy Treat with Na restriction, ACE inhibitors, Beta blockers, diuretics, digoxin, ICD, heart transplant

Answer 387

commonly a beta-myosin heavy chain mutation. you see myofibrillar disarray and fibrosis. huge heart with marked ventricular hypertrophy, often septal predominance. abnormal diastole because the heart cannot fill (the chambers are small from all the extra muscle). Hypertrophic Obstructive cardiomyopathy HOCM: a subset of HCM, with asymmetric septal hypertrophy and systolic anterior motion of mitral valve, causing an outflow obstruction, which causes dyspnea/syncope. you have a dynamic LVOT obstruction that is worsened with decreased LV volume, so you need to AVOID vasodilators (decrease SVR, decrease after load, so decrease LV vol) and AVOID diuretics (decrease preload and outflow obstruction) exercise-induced syncope (due to outflow obstruction) and sudden death (ventricular arrhythmia) in young athletes findings: S4, systolic murmur (louder standing) may see mitral valve regurg due to impaired mitral valve closure.

Answer 388

concentric LVH: LV wall is very thick and muscle-y caused by pressure overload so the LV has to work extra hard to pump during systole, so it's going to get bigger: chronic HTN (increased LV after load) aortic stenosis pts may develop diastolic dysfunction with LA enlargement and CHF 2/2 impaired ventricular compliance and filling. ``` eccentric LVH: LV is very big and dilated caused by volume overload, so the LV has to dilated to accommodate the extra volume: aortic or mitral regurg myocardial infarction dilated cardiomyopathy ```

Answer 389

``` concentric LVH: LV is very thick and muscle-y caused by pressure overload so the LV has to work extra hard to pump, so it's going to get bigger: chronic HTN aortic stenosis ``` ``` eccentric LVH: LV is very big and dilated caused by volume overload, so the LV has to dilated to accommodate the extra volume: aortic or mitral regurg myocardial infarction dilated cardiomyopathy ```

Answer 390

``` NF-1: AKA von Recklinghausen disease gene mutation in NF1 tumor suppressor gene, that normally codes neurofibromin protein on Chromosome 17 clinically, you see: Café-Au-Lait spots multiple neurofibromas Lisch nodules ``` ``` NF-2: AKA central neurofibromatosis gene mutation on NF2 tumor suppressor gene, that normally codes the merlin protein on Chromosome 22 clinically: bilateral acoustic neuromas ```

Answer 391

mutation of VHL on chromosome 3 (3 letters = chr 3) characterized by renal cysts, renal cell carcinomas, and hemangioblastomas of the cerebellum and retina

Answer 392

mutation of VHL on chromosome 3 (3 letters = chr 3) characterized by renal cysts, renal cell carcinomas, and hemangioblastomas of the cerebellum and retina

Answer 393

Migraine: often unilateral pulsatile and throbbing auras, photophobia, photophobie, nausea ``` Cluster: onset during sleep begins behind the eye! excruciating, sharp, and steady pain causes sweating, facial flushing, nasal congestion, lacrimation, and pupillary change ``` Tension: occurs under stress "band like pattern" around head (bilateral) described as dull, tight, and persistent muscle tenderness in head, neck, and shoulders

Answer 394

Gastrin: increase gastric H+ secretion released from G cells (antrum, duodenum) Somatostatin: decrease secretion of most GI hormones released from D cells (pancreatic islets, gut mucosa) CCK: increase pancreatic enzyme and HCO3 secretion released from I cells (Small intestine) Secretin: increase pancreatic HCO3 secretion decrease gastric H secretion released from S cells (small intestine) GIP: increase Insulin release decrease gastric H+ secretion released from K cells (small intestine) Motilin: increase GI motility released from M cells (small intestine) G cells are the only ones that increase gastric acid secretion. MD KISs cells all decrease gastric acidity somehow

Answer 395

adenocarcinoma of gallbladder cholangiocarcinoma risk is increased in fibrotic disease of bile ducts (PSC, chronic infection)

Answer 396

adenocarcinoma of gallbladder cholangiocarcinoma risk is increased in fibrotic disease of bile ducts (PSC, chronic infection)

Answer 397

decrease in FFA oxidation (breakdown) 2/2 excess NADH production by the 2 major alcohol metabolism enzymes: alcohol dehydrogenase and aldehyde dehydrogenase

Answer 398

decrease inf FFA oxidation (breakdown) 2/2 excess NADH production by the 2 major alcohol metabolism enzymes: alcohol dehydrogenase and aldehyde dehydrogenase

Answer 399

most common cardiac tumor in adults most often in LA described as "ball valve" obstruction in LA (multiple syncopal episodes). may hear early diastole "tumor plop" rumble murmur; positional dyspnea- worse with sitting, improves laying down Histology: scattered cells within a mucopolysaccharide stroma, abnormal vessels, and stroma

Answer 400

postprandial epigastric pain and associated food aversion/weight loss in the setting of generalized atherosclerosis not relieved by antacids. caused by diminished blood flow to intestine after meals

Answer 401

postprandial epigastric pain and associated food aversion/weight loss in the setting of generalized atherosclerosis not relieved by antacids. caused by diminished blood flow to intestine after meals

Answer 402

bovis in the blood = cancer in the colon gram + cocci colonizes GI formerly staph gallolyticus

Answer 403

cholera-like from ETEC, an E coli that produces heat labile (LT) and heat stable (ST) toxins LT activates adenylate cycles leading to increased cAMP ST activates guanylate cycles leading to increased cGMP (eL Agua de San Guan)

Answer 404

it's a Flavivirus, ssRNA+ transmitted by female mosquitos also an arbovirus (arthropod-borne virus) ``` presents with: encephalitis meningitis flaccid paralysis seizures coma (swan birds in the Sketchy West Nile/Hep C River) also fever and rash ``` diagnose with CSF anti-WNV antibodies (PCR often not needed)

Answer 405

BOAR all are RNA viruses Bunyavirus Orthomyxovirus (influnza viruses) Arenavirus Reovirus

Answer 406

Staph aureus and the satellite phenomenon provide Factor X and 5 (Hematin and NAD+) for growth

Answer 407

often caused by cirrhosis it arises from increased resistance to portal flow at the hepatic sinusoids. chronic portal HTN leads to dilation of small, pre-existing vascular channels between the portal and systemic circulations. Esophageal varices arise from anastomoses from L gastric vein and esophageal vein Anorectal varices arise from superior rectal vein and middle and inferior rectal veins caput medusae arise from par umbilical veins and superficial and inferior epigastric veins

Answer 408

often caused by cirrhosis it arises from increased resistance to portal flow at the hepatic sinusoids. chronic portal HTN leads to dilation of small, pre-existing vascular channels between the portal and systemic circulations. Esophageal varices arise from anastomoses from L gastric vein and esophageal vein Anorectal varices arise from superior rectal vein and middle and inferior rectal veins caput medusae arise from par umbilical veins and superficial and inferior epigastric veins

Answer 409

V1, V2 = LAD V3, V4 = distal LAD V5, V6 = LAD or LCX "I, avL = Lateral (LCX)" "II, III, avF = inFerior (RCA)" V7- V9; ST depression in V1-V3 with tall R waves = PDA

Answer 410

Increased osteoclastic activity, causing elevated serum Ca. This has neg feedback to decrease PTH levels. low PTH means decreased renal resorption, so you have hypercalciuria. The combo of hypercalcemia and light chain cast nephropathy cause progressive renal failure, leading to low 1,25-(OH)2-VitD MM is a monoclonal plasma cell (fried egg) that arises the marrow and produces large amounts of IgG or IgA. Associated with: primary amyloidosis (AL) punched-out lytic bone lesions M spike on serum protein electrophoresis Ig light chains in urine (Bence Jones Protein) Rouleaux formation (stacked RBCs) Numerous plasma cells w/ "clock face" chromatin and Ig inclusions ``` think CRAB: hyperCalcemia (constipation) Renal involvement (high serum protein) Anemia (fatigue) Bone lytic lesions/ Back pain ``` Multiple Myeloma = Monoclonal M protein spike

Answer 411

resorbs all glucose and AAs, and most of all the other anions and water. It resorbs uric acid too. this is all isotonic absorption. It generates and secretes NH3, which acts as a buffer for secreted H+ PTH acts here to facilitate Phosphate excretion Ang2 acts here to stimulate the Na/H+ exchange pump, which causes high Na, H2O, and HCO3 reabsorption. 65-80% of Na is reabsorbed here

Answer 412

Thin Descending: passively reabsorbs H2O, but is impermeable to Na, so this is the concentrating segment. Urine is most concentrated at the most distal tip Thick Ascending loop: this uses the Na/K/2Cl co transporter to reabsorb all of those. It indirectly reabsorbs Mg and Ca through a positive lumen potential (2/2 K backleak.) it is impermeable to H2O, so it makes urine less concentrated as it ascends. 10-20% of Na is reabsorbed here.

Answer 413

reabsorbs Na and Cl. this makes urine fully dilute (hypotonic, lowest osmolality!!). PTH acts here to stimulate the Ca/Na exchange and cause Ca reabsorption. 5-10% of Na is reabsorbed here. it is H2O impermeable in the early part, but is variable in the later portion 2/2 vasopressin

Answer 414

aldosterone regulates the exchange of Na for K and H+ excretion (reabsorbs Na and water). principal cells: K secretion alpha-intercalated cells: HCO3/Cl exchanger activity ADH acts at the V2 receptor, inserting aquaporin H2O channels on the apical side, causing more water reabsorption and therefore volume retention 3-5% Na is reabsorbed here

Answer 415

Charcot Triad of MS is a SIN: Scanning speech Intention tremor, Incontinence, INO Nystagmus Incontinence is from UMN lesion so bladder does not distend/relax properly due to loss of descending inhibitory control from the UMN, causing bladder hypertonia, increased urinary frequency, and urge incontinence INO is caused from an MLF lesion (MLF in MS). the MLF is unable to coordinate eyes moving in the same horizontal direction, so you have nystagmus of the abducting eye with impaired adduction of the other eye. (if you have Right INO, that means the R eye is paralyzed and cannot adduct) Convergence is normal though!

Answer 416

JG cells (modified SM cells within the afferent arterioles) will undergo hyperplasia to increase levels of Ang2 and Aldosterone to increase blood volumes.

Answer 417

eRPF can be estimated using PAH clearance. PAH has some of it freely filtered from blood to glomerular capillaries in Bowman's space, but most of it is secreted from blood into tubular fluid by carrier-mediated proteins (capable of being saturated) into the PCT. this means nearly 100% of PAH will be excreted that enters the kidney. PAH is not reabsorbed anywhere. eRPF underestimates the true RPF slightly. you can calculate this by: eRPF = PAH clearance = [(urine [PAH] x urine flow rate) / (plasma [PAH])]

Answer 418

prostate undergoes hyperplasia, so it's rubbery Bladder wall hypertrophies to increase its contractile force Ureters etc dilate to hydronephrosis Renal parenchyma: atrophy and scarring due to reflux of urine and damage of renal tissue

Answer 419

glomerular basement membrane thickening and increased mesangial matrix deposition, leading to: Kimmelstiel-Wilson Nodules- areas of hyaline arterioloscerlosis and nodular glomeruloscerlosis (large pink clearings). These indicate irreversible glomerular damage. They will have a lamellate appearance, eosinophilic on H&E stain, and PAS+ progressive proteinuria with eventual nephrotic syndrome and renal failure.

Answer 420

Calcium: calcium oxalate stones, radiopaque shaped like envelope (square w/ X) or dumbbell they're most common (80%). can result from hypcitraturia, ethylene glycol ingestion, Vit C abuse, or malabsorption. calcium phosphate stones, radiopaque shaped like wedge-shaped prism Ammonium Magnesium Phosphate: AMP high pH!!! (the bugs de-acidify the environment) radiopaque shaped like coffin lid AKA struvite 2/2 urease (+) bugs, incl Proteus, Staph saphrophyticus, and Klebsiella, that hydrolase urea to NH3 and alkalinize the urine. Commonly form staghorn calculi. Uric acid: radiolUcent shaped like rhomboids or rosettes, yellow or brown at risk in low urine vol's, arid climates, and acidic pH. Strong association with gout, or increased cell turnover (leukemia). ``` Cystine: radiolucent shaped like hexagon- flat Hereditary condition wehret Cystine-reabsorbing PCT transporter loses function causing cystinuria. Transporter defect also causes decrease in Ornithine, Lysine, and Arginine (COLA!)- aminoaciduria!. "SIX-tine stones have SIX sides" ```

Answer 421

type 2 hypersensitivity where you form anti-GBM antibodies (type 4 collagen). these antibodies target the pulmonary alveoli and the glomerular BM, causing hemoptysis and hematuria with a linear IgG deposition

Answer 422

(glomerular capillary hydrostatic pressure - Bowman capsule hydrostatic pressure) - (glomerular capillary oncotic pressure - Bowman capsule oncotic pressure) (Pc - Pi) - (PIc - PIi)

Answer 423

nonlinear when GFR is normal, it takes a large change in GFR to affect/increase Creatinine when GFR is low/small, a small decrease in GFR causes a large increase in Creatinine

Answer 424

enlarged, hypercellular glomeruli on LM "lumpy bumpy" granular deposits of IgG and C3 on immunofluorescence electron-dense deposits on the epithelial side of the BM on EM (sub-epithelial humps) Goodpasture syndrome: linear IgG and C3 deposits on IF; Crescent on LM (fibrin) pulmonary symptoms

Answer 425

AKA IgA nephropathy presents as recurrent, self-limited painless hematuria shortly after an URI. Kidney bx will show mesangial IgA deposits. PSGN occurs 1-3 weeks after illness and is not recurrent

Answer 426

amyloidosis

Answer 427

minimal change disease and FSGN

Answer 428

disorder of Type 4 collagen, so you have lamellated BM (type 4 on the floor) with irregular thinning and thickening (basket-weave appearance) associated with hearing loss and ocular abnormalities "can't see, can't pee, can't hear a bee"

Answer 429

Membranous glomerulonephropathy: diffuse GBM thickening, with "spike and dome" from sub-epithelial immune complex deposits (granular IF) this is the nephrotic (less common) presentation of SLE!!! associated with IgG4 antibodies to PLA2R (on podocytes, so don't jump to MCD or FSGN) Membranoproliferative glomerulonephropathy: immune complex deposition with granular IF causes "tram-tracking" because the deposit causes GBM splitting by mesangial cells Type 1: sub endothelial Type 2: basement membrane (sub-epithelial is MGN above)

Answer 430

DM: NEG of the vascular BM leads to hyaline arteriolosclerosis characterized by Kimmelstiel-Wilson nodules Systemic amyloidosis: kidney is most commonly involved organ deposits in mesangium apple green birefringence on Congo Red stain

Answer 431

Goodpasture: affects lung, kidney linear IF ``` Wegener: affects lung, kidney, and nasopharynx C-ANCA RPGN (C disease) ```

Answer 432

microscopic polyangiitis Churn-Strauss: granulomatous inflammation eosinophilia associated with Asthma

Answer 433

vesicoureteral reflux, causing retrograde reflux and inflammation (posterior urethral valves are a malformation of Wolffian ducts and only occur in Males)

Answer 434

most common nephrotic syndrome in children increased glomerular capillary permeability causes massive protein (albumin) loss in urine. Hypoalbuminemia reduces plasma oncotic pressure, which causes fluid shift to interstitial spaces, giving you edema. Low oncotic pressure also triggers increased lipoprotein production in the liver (hyperlipidemia and hyperlipiduria)

Answer 435

large eosinophilic casts composed of Bence-Jones proteins

Answer 436

child recovering from URI gets abdominal pain, arthralgias, buttock palpable purpura!!! (vs HUS/TTP with Shiga-like toxin or neuro symptoms), and hematuria it's an IgA-mediated hypersensitivity vasculitis, so it's associated with IgA nephropathy (Berger disease)

Answer 437

FF = GFR / RPF RPF is better than RBF because RBF includes volume occupied by RBCs RPF = RBF x (1-Hct) so at 1 L/min and a Hct of 0.5, the RPF would be 0.5L.min then if you know the GFR, calculate the FF

Answer 438

Inulin clearance can be used to estimate GFR and calculate filtration rate of a freely filtered substance when its plasma concentration is known. Inulin is freely filtered and neither reabsorbed nor secreted (PAH is partially secreted) Creatinine clearance is an approximate measure of GFR, but it slightly overestimates GFR because it's moderately secreted by renal tubules.

Answer 439

Albumin glucose only shows up in urine after serum glucose exceeds 200 (saturation), and it does not correlate with degree of renal damage in DM

Answer 440

HUS: common cause of ARF in children triad of: microangiopathic hemolytic anemia, thrombocytopenia, and AKI most develop 2/2 diarrheal illness (shiga toxin organisms, including E Coli and Shigella) you see schistocytes 2/2 the intravascular hemolysis. HUS is on the same spectrum as TTP: caused by defect in ADAMTS13, causing a defect in degrading vWF multimers. Large multimers causes plt adhesion, aggregation, and thrombosis. you still see schistocytes 2/2 intravascular hemolysis, and elevated LDH. Symptoms include a pentad: neuro, renal, fever, thrombocytopenia, microangiopathic hemolytic anemia both of these are plt plug formation disorders, not coagulation disorders, so you have normal PT/PTT (vs DIC activating the coagulation cascade) but prolonged bleeding time

Answer 441

hypercellular glomeruli with hematuria, proteinuria, and urine RBC casts, usually in a child after an illness ``` lab findings: ASO titers anti-DNase B low C3 (normal C4) presence of cryoglobulins ```

Answer 442

renal papillary necrosis sloughing of necrotic renal papillae, causing acute gross hematuria and proteinuria with flank pain. may be triggered by recent infection or sickle cell, acute pyelonephritis, NSAIDs, or DM. ``` SAAD papa with papillary necrosis: Sickle cell Acute pyelonephritis Analgesics (NSAIDs) DM ```

Answer 443

malignant hyperthermia severe hyperkalemia, esp in pts with burns, myopathies, crush injuries, and denervation bradycardia from parasympathetic stimulation or tachycardia from sympathetic effects

Answer 444

spironolactone and eplerenone. Aldosterone normally acts on principal and intercalated cells of the renal collecting tubules to cause resorption of Na and water, while losing K and H+

Answer 445

acute rise in serum Cr by blocking Angiotensin-mediated efferent arteriole constriction. this leads to a reduction in Filtration fraction! for pts dependent on the efferent arteriole construction to maintain their renal perfusion (renal artery stenosis), ACE inhibitors will precipitate ARF

Answer 446

causes defunctionalization of afferent pain fibers and depletion of substance P. initial application burns, but chronic exposure leads to reduced pain transmission. consider in someone with post-herpetic neuralgia

Answer 447

GABA-b receptor agonist it's used to treat muscle spasticity 2/2 brain and spinal cord disease, like Multiple Sclerosis. you could also you Tizanidine, an alpha-2 agonist (to-Christine on the massage chair in the phantom of the opera sketchy)

Answer 448

NMS = neuroleptic malignant syndrome, and it can be caused by lots of different drugs, including anti-psychotics. you can treat this with Dantrolene, which inhibits Ca ion release from SR on skeletal muscles to decrease contraction and decrease heat production. you can also use Dantrolene in Malignant hyperthermia 2/2 inhaled anesthetics. That's caused by defective RyR receptor causing skeletal muscle hypersensitivity to inhaled anesthetics by releasing excess Ca release and excess ATP uptake --> heat production

Answer 449

Anorexia nervosa: excessive dieting, exercise, or binge eating/purging; intense fear of gaining weight; loss of pulsatile GnRH BMI <18.5 Bulimia nervosa: Binge eating with inappropriate compensatory behaviors (self-induced vomiting, laxatives, fasting, excessive exercise) over-evaluation of body image BMI often normal Binge Eating Disorder: excessive eating without inappropriate compensatory behaviors

Answer 450

mosquitos carry sporozoites in their saliva (bloody mushrooms) mosquitos bite human host, sporozoites travel to liver, and mature to trophozoites then schizont, leading to rupture of hepatocyte and release of merozoites merozoites go on to infect RBCs lifecycle continues on to trophozoites, schizont, merozoite, etc. immature schizont has a ring form merozoite can also form a gametocyte, which helps infect mosquitos when they bite the infected host

Answer 451

Merocrine: cells secrete watery fluid via exocytosis with no loss of cytoplasmic membrane examples incl salivary glands, eccrine sweat glands, and apocrine sweat glands Apocrine: cells secrete via membrane-bound vesicles ex is mammary gland Holocrine: cell lysis releases entire contents of the cytoplasm and cell membrane examples incl sebaceous glands (ACNE) and meibomian glands (eyelid)

Answer 452

Dermatitis herpetiformis rash on bilateral extensor surfaces. IgA antibodies against gliadin, giving you increased intestinal intraepithelial lymphocytes. the IgA deposits at the dermal papillae tips. It's IgA because it's mucosal associated. IgG mediated skin disruption is seen in pemphigus vulgarism and bullis pemphigoid.

Answer 453

BRAF, a protein kinase for signaling pathways, causing increased activation and cell growth and metastasis.

Answer 454

hypertrophic: parallel collagen synthesis confined to the borders of the original wound; possible spontaneous regression keloid: excessive, disorganized collagen synthesis from high TGF-beta in fibroblasts. extends beyond wound with claw-like projections. frequently recurs

Answer 455

Hemostasis: 0-1 days vasoconstriction, plt aggregation, fibrin deposition Inflammatory: up to 3 days after wound plts, PMNs, and macrophages: clot formation, leaky vessels and neutrophil migration to tissue; macrophages clear debris 2 days later Proliferative: 3 days - 3 weeks fibroblasts, myofibroblasts, endothelial cells, keratinocytes, and macrophages: deposition of granulation tissue and type 3 collagen (vascular); angiogenesis, epithelial cell proliferation, dissolution of clot, wound contraction (mediated by myofibroblasts accumulating at edges) keloids result from excessive, disorganized collagen formation Remodeling/Maturation: 3 weeks- months: fibroblasts: type 3 collagen replaced by type 1 collagen, to increase tensile strength of tissue

Answer 456

normally you'd treat with acyclovir, a guanosine nucleoside analog, but it requires thymidine kinase (virus specific) as its rate-limiting step to get its first Phosphate group added. If your strain is resistant and has altered/lacking viral phosphorylating enzymes, you Cidofovir which does not require phosphorylation by the viral kinase (Cid clearing up the blockage at the airport in sketchy) or Foscarnet- no phosphorylation necessary. it is a pyrophosphate analog that can chelate Ca and promote nephrotoxic renal Mg wasting, which can cause seizures both of these directly inhibit viral DNA polymerase

Answer 457

epidermal accumulation of edematous fluid in intercellular spaces causes allergic dermatitis

Answer 458

one option is topical Vitamin D analogs, including calcipotriene, calcitriol, and tacalcitol, which bind to the Vit D receptor and inhibit keratinocyte proliferation and stimulate keratinocyte differentiation. psoriasis presents with slivery scaling on a salmon background. it has acanthosis with parakeratotic scaling (nuclei in stratum corneum) , Munro micro abscesses (neutrophil clusters), decreased stratum granulosum. Also has Auspitz sign (pinpoint bleeding) if you take off the scale because the dermal papillae are so close to the surface.

Answer 459

IgE- independent mast cell degranulation. common after several med administrations. IgE-dependent mast cell degranulation is typically associated with environmental exposures like food or stings.

Answer 460

Pyrimidine dimers are formed in the DNA. They are recognized by an endonuclease complex that repairs via nicking the damaged strand on both sides of the pyrimidine dimer. the damage segment is excised, and a replacement DNA is synthesized by DNA polymerase. this is Nucleotide excision repair, NER. NER is impaired in xeroderma pigmentosum, and causes severe photosensitivity and early skin cancers

Answer 461

this causes double-strand DNA breaks and is repaired via non homologous end joining

Answer 462

pemphigus vulgaris: autoantibodies against desmosomes/desmoglein in the stratum spinosum, which is supposed to have the "spikes" of intercellular connections. these blisters will be superficial and flaccid. they commonly present on oral mucosa, and spread laterally when pressured. "if you're a really vulgar person, you're more likely to be superficial and shallow" bullous pemphigoid: autoantibodies against hemidesmososmes connecting the stratum basalis to the BM. this causes separation of epidermis and dermis, causing a thick, tense blister. mucosal involvement is rare. "bulls are deep and thick animals"

Answer 463

hives. wheals that form 2/2 IgE-mediated mast cell degranulation. edema in the superficial dermal layer

Answer 464

epidermal atrophy with flattening of the rete edges from UVA photoaging. decreased collagen fibril production increased degradation of collagen and elastin in dermis

Answer 465

rare malignancy of head, neck, and breast areas. elderly, sun-exposed areas associated with: radiation therapy, post mastectomy lymphedema described as firm violaceous nodule on ipsilateral side

Answer 466

intranuclear inclusions in keratinocytes and multinucleated giant cells, which is a positive Tzanck smear microabsessces at the dermal papillae tips is IgA-mediated dermatitis herpetiformis associated with Celiac

Answer 467

psoriatic arthritis, nail pitting, and uveitis

Answer 468

``` cherry: hemangioma of elderly do not regress increase in frequency with age "don't give cherries to babies because they'd choke on the pit" ``` strawberry: hemangioma of infancy grows rapidly then regresses spontaneously by 5-8yo.

Answer 469

vitiligo: autoimmune destruction of melanocyte cells "absence of melanocytes in skin" albinism: normal melanocyte cells with low melanin production 2/2 low tyrosinase activity or defective tyrosine transport. "poor melanin production"

Answer 470

premalignant skin lesion 2/2 sun exposure. small, rough, erythematous or brown spots with atypical keratinocytes, parakeratosis, and hyperkeratosis. described like "sandpaper" so pt may be rubbing it a lot. they do not invade dermis but may transform to squamous cell carcinoma

Answer 471

small red-blue lesion under nail bed originates from modified SM cells that control thermoregulatory functions of dermal glomus bodies

Answer 472

cell-mediated inflammatory disorder of skin w/ target lesions. most commonly seen in immunocompetent HSV pts. immunocompromised pts might present with disseminated herpes simplex infection

Answer 473

spirochete bacteria (galaxy) visualized on dark microscopy. "screen" with VDRL confirm with FTA-Ab (a spirochete antibody on UWORLD) primary: painless genital ulcer/chancre and ischemic necrosis (takes out nerves- no pain) secondary: "systemic" solar system infection rash on palms and soles, but also develops everywhere (astronaut's red gloves and boots) condyloma lata (planet with lots of bumps; vs HPV's condyloma acuminata with wart-like) visualize spirochetes w/ dark field microscopy (kid telescopes) tertiary: gummas (soft growths with firm necrotic centers that can occur anywhere, like the moon) aortitis, esp ascending aorta, which can lead to aortic aneurysm pathologically- "tree barking"; syphilis destroys the vasa vasorum that supplies blood to the aorta tabes dorsalis- demyelination of dorsal roots and dorsal columns (damaged columns) Argyll Robertson pupils: react to accommodation but not to light (Argyle wearing Robert is an accommodating greeter that does not react to the kid's flashlight); AKA prostitute's pupil Congenital syphilis: constellation of symptoms in children- saber shins (anterior bowing of tibia) and saddle shaped nose (warrior + horse constellation) Hutchinson teeth and Mulberry molars (shivering kids) deafness (earmuffs) treat: Penicillin, always! Jarisch-Herxheimer reaction- may occur hours after tx; dying spirochetes that release cytokines (good indicator that tx is working) giant ball of fire and ice in space- fevers and chills

Answer 474

tetracyclines: teeth staining chloramphenicol: "gray baby" syndrome trimepthoprim/sulfamethoxazole: neural tube defects ahminoglycosides: ototoxicity, vestibulotoxicicty

Answer 475

stimulate RBC production and help tx anemia of chronic kidney disease, but may worsen HTN and thromboembolic events

Answer 476

Furosemide! | the seashell in the middle of the looping roller coaster

Answer 477

ACE inhibitors or ARBs will block Ang2, which is necessary for normal renal development. these will cause anuria, oligohydramnios, pulmonary hypoplasia, limb contracture, and calvarium defects

Answer 478

``` misoprostol: PGE1 agonist ("Extreme sports" missing the ball) ``` Mifepristone: progesterone antagonist glucocorticoid antagonist (stone hinge) Methotrexate: folic acid antagonist

Answer 479

disulfiram-like reaction when combined with alcoholic due to acetaldehyde accumulation disulfiram inhibits acetaldehyde dehydrogenase, which is supposed to convert acetaldehyde to acetic acid

Answer 480

2/2 prenatal lithium exposure (schizophrenic mother) causes apical displacement of tricuspid valve leaflets, decreased RV volume, and RV atrialization (snowman in Lithium sketchy)

Answer 481

dysmorphic facies, growth retardation, and CNS abnormalities dysmorphic facies incl thin vermilion border, smooth philtrum, small palpebral fissures, and microcephaly also VSD, PDA, ASD, and tetralogy of Fallot

Answer 482

remove introns from pre-mRNA during intranuclear processing. snRNPs are an important protein that make up spliceosomes for LMN function. mutations in SMN1 gene cause impaired assembly of snRNPs in LMN, causing infantile flaccid paralysis called spinal muscular atrophy.

Answer 483

Bile acid resins: cholestyramine (lobster): main goal is to prevent intestinal reabsorption of bile acids. this forces the liver to make more. you have a slight increase in TG, but a decent reduction in LDL because the liver has more LDL receptors. you risk GI upset, decreased intestinal absorption of other drugs and vitamins, including Statins, so give them 4 hrs apart Ezetimibe: Z shaped eel: it prevents cholesterol absorption at the brush border in the intestine. this restricts the liver's access to cholesterol, so it has to make its own. You increase LDL receptors to decrease systemic LDL. you risk a rare increase in LFTs, and diarrhea

Answer 484

AKA Vit B3: Loch Niacin monster it's main role is to inhibit lipolysis in adipose tissue. it reduces hepatic VLDL synthesis and secretion, so it therefore lowers LDL. it's also the best lipid drug to raise HDL (submarine on Nessy's back) you risk red, flushed face mediated by PGE, so you can reduce this by using w/ NSAIDs. you risk liver damage and elevated LFTs, so you must monitor LFTs. you risk hyperglycemia, and hyperuricemia, so give with Allopurinol.

Answer 485

involved in transamination reactions! (AST and ALT) these occur between an AA and an alpha-keto acid. also in decarboxylation and glycogen phosphorylase rxns. it synthesizes cystathionine, heme, niacin, histamine, and neurotransmitters (serotonin, EPI, NE, Dopamine, GABA)

Answer 486

mutation in Janus Kinase 2 (JAK2), which is a cytoplasmic tyrosine kinase. this causes tyrosine kinase activity, and activation of signal transducers and activators of transcription (STAT) proteins, the JAK-STAT signaling pathway these are overproduction of myeloid cells

Answer 487

converts superoxide oxygen to H2O2 in the production of ROS. H2O2 can then be converted H2O for detox via catalase/gluathione peroxidase. NADPH oxidase catalyzes the reduction of molecular oxygen to superoxide free radicals, aiding in bacterial destruction via phagocytes. deficiency in NADPH oxidase cause CGD and pts are unable to destroy catalase-positive organisms.

Answer 488

primary carnitine deficiency: it impairs fatty acid transport from the cytoplasm into the mitochondria, which prevents beta-oxidation of fatty acids into acetyl CoA (degradation of FAs to be used for energy in TCA cycle or ketone bodies.) this leads to cardiac and skeletal myocyte injury (lack of ATP from citric acid cycle) and impaired ketone body (acetoacetate!) production by the liver during fasting. pts have weakness, hypotonia, hypoketotic hypoglycemia, and cardiomyopathy. Medium chain acyl-CoA dehydrogenase deficiency, MCAD deficiency, presents similarly: disorder of fatty acid beta-oxidation, so you have accumulation of carnitines in the blood and hypoketotic hypoglycemia. treat by avoiding fasting, and giving glucose during illness. Fasting pts are unable to oxidize their FAs for energy in the TCA cycle, nor are they able to turn them into ketone bodies.

Answer 489

Glutamine! it's converted to glutamate, then alpha-ketoglutarate, then glucose in renal tubules. in the process, it produces NH3 as a byproduct, and CO₂ as a byproduct. CO₂ generates H+ ions, which combine with NH3 in the tubular lumen to make NH4+ which can be excreted in the urine as excess acid. Arginine is a urea cycle intermediate that helps remove Nitrogenous waste products (NH4+) from blood. Hepatic! metabolism of arginine results in production of urea and ornithine.

Answer 490

effect modification: when the effect of an exposure on an outcome is modified by another variable. it can be identified using stratified analysis (using different subgroups), and the different groups will have different measures of association. For example: smoking status + new drug (exposure) effect on DVT (outcome) Among smokers, there was a difference in developing DVT outcomes with the new drug. Among non-smokers, there was no difference in development of DVT in the groups taking the drug or not taking the drug. Different measures of association! Confounding: stratification usually reveals no significant difference between the strata. For example: Elementary school age is a confounder that muddles the association between shoe size and intelligence. but if you analyze the younger and older kids separately (stratify based on the confounder), the association between shoe size and intelligence disappears.

Answer 491

1 g protein = 4 kcal 1 g carb = 4 kcal 1 g fat = 9 kcal 1 g alcohol = 7 kcal

Answer 492

``` Alanine Valine Leucine Isoleucine Phenylalanine Tryptophan Methionine Proline Glycine ``` they play an important role in making transmembrane proteins' domains of non polar, hydrophobic areas

Answer 493

silent: same AA is made; change in the wobble position (genetic code is degenerate) missense: changed AA Nonsense: early stop codon (Stop the nonsense!) stop codons are UAA, UAG, UGA Frameshift: deletion not divisible by 3 Splice site: retained intron

Answer 494

Brief psychotic disorder: lasting <1 month, usually stress related Schizophreniform disorder: 1-6 months Schizophrenia: >6 months Schizoaffective disorder: >2 weeks of hallucinations/delusions alone + mood with hallucinations/delusions

Answer 495

Depression with psychotic features: Mood alone + mood and schizophrenia Schizoaffective disorder: schizophrenia alone + mood and schizophrenia

Answer 496

Bipolar 1: 1 = most extreme; mania requires at least 1 manic episode, and then +/- hypomania or depressive episode Bipolar 2: 2 = 2 different presentations required presence of a hypomanic AND a depressive episode so if you ever have a manic episode, that's automatically Bipolar 1. it can occur with our without psychotic features (delusions, hallucinations). depressive episodes are not required for a bipolar 1 diagnosis.

Answer 497

make sure the pt has never had a manic episode before to rule out bipolar disorder. this is different from psychosocial history Antidepressants can ppt mania

Answer 498

AV node refractory period the atrium is sending chaotic signals but the AV node's refractory period makes sure the majority of atrial impulses never reach the ventricles

Answer 499

fast --> slow: purkinje Atrial muscle Ventricular muscle AV node "Park At Venture Ave"

Answer 500

narrow part of submucosa, because that's where the (absent) nerve plexuses should be

Answer 501

an excitatory NT (glutamate) binds to postsynaptic dendrite, which opens ligand-gated Na channels Na influx, membrane depolarization impulse travels to axon hillock, which contains a large number of voltage-gated Na channels when the axon hillock becomes depolarized, the AP is triggered an propagates along axon via steady influx of Na ions. when the AP reaches the axon terminal, Voltage gated Ca channels open and allow influx of Ca this is essential for release of NT vesicles into the synaptic cleft

Answer 502

Tropomyosin prevents cross linking between actin and myosin. Ca is released from SR via RyR and binds to troponin, which removes tropomyosin. this exposes the myosin cross-bridge-ADP-phosphate complex to bind to actin. the ADP-Phosphate complex is released after the myosin cross bridge binds to actin a new ATP is required for detachment of the myosin cross bridge from actin (without the ATP, the muscle will remain contracted- rigor mortis). Hydrolysis of the bound ATP --> ADP causes the myosin head to be in a "cocked" high energy position for the next contraction

Answer 503

A band: All of myosin (includes overlapped actin) "All of me; length is Always the same" M line: center of the A band myosin anchors to structural elements "Mom is my center anchor" I band: only actin (only thin) "I am only active when I am alone" H band: only myosin (only thick) "only Heavy" H and I both decrease during contraction: Hi Z line: structural proteins for actin binding splits the I band, and typically shows up as a thick line on the imaging

Answer 504

autoimmune plt destruction normal Hct, normal WBC low platelets normal Fibrinogen normal PT

Answer 505

widespread activation of coagulation cascade, so there would be low clotting factors 2/2 consumption low plts low fibrinogen or other clotting factors (Factor 1) prolonged PT, PTT

Answer 506

intrinsic coagulation defect. platelet function is impaired, but platelet count is normal. NORMAL plt count prolonged bleeding normal PT prolonged PTT because vWF acts to carry Factor 8)

Answer 507

ER and PR positivity indicate favorable response to tx with: Tamoxifen Aromatase inhibitor HER2 over expression correlates with an aggressive tumor that responds to anti-HER2 monoclonal antibody Trastuzumab

Answer 508

decrease LV after load the amount of blood that flows forward (to the aorta) is determined in part byLV after load. if you decrease after load, resistance to blood flow into the aorta is reduced, so blood is diverted away from LA

Answer 509

if your PT fails to correct after you give Vitamin K factor 7 has the shortest half life and is the first to be abnormal. this is often due to underlying liver cirrhosis/alcholism because the liver synthesizes clotting factors 2, 7, 9, and 10

Answer 510

during healing, excessive matrix metalloproteinase activity and myofibroblast accumulation in the wound margins cause contracture. this produces deformities of the wound and surrounding tissues. keloids are excessive collagenous scar tissue that deposits beyond wound margins

Answer 511

usually occurs after repetitive activity characterized by sharp sternal pain that's reproducible with palpation and worsened with movement, changes in position, or deep inspiration vs pericarditis, which follows a URI, worse when lying flat, friction rub

Answer 512

OSA presents as excessive daytime sleepiness and signs of upper airway obstruction (snoring, gasping) associated with systemic HTN, so you're at risk for developing pulm HTN and RHF

Answer 513

N/V, confusion, dizziness, tinnitus, fever, tachypnea. 2 different acid/base abnormalities: respiratory alkalosis occurs first, as the respiratory center is stimulated so your ventilation is increased (blow off CO₂ and acid) anion gap metabolic acidosis (mudpileS) develops ~12 hours later as high ASA conc's increase lipolysis and inhibit TCA cycle. this results in accumulation of organic acids in blood (ketoacids, lactate, pyruvate), which bind to HCO3, increasing the calculated anion gap.

Answer 514

normal spitting: inspiration decreases intrathroacic pressure, increasing venous return, which increases RV stroke volume, causing the RV to ejection time to be longer, so the P2 noise will be longer after the A2. Wide splitting: seen with delayed RV emptying (pulmonic stenosis) cause delayed delayed P2, which is more exaggerated during inspiration. this is an exaggeration of normal splitting Fixed splitting: heard in ASD! ASD causes a L--> R shunt, which increases RA and RV volumes. Regardless of breath, P2 closure is greatly delayed. think about paradoxical (artery) embolisms here Paradoxical splitting: heard when aortic valve closure is delayed (aortic stenosis), so you hear P2 before A2. on inspiration, the P2 is still delayed (like in normal splitting), so the P2 closes just before A2, thereby "paradoxically" eliminating the split that is normally heard on expiration.

Answer 515

little bigger than RBCs "clock face" chromatin distribution and eccentric nucleus, abundant RER, and well-developed Golgi. It think you can see them with a prominent purple nucleus and then a small slit? malignant plasma cells are seen in AL amyloidosis and Multiple Myeloma

Answer 516

lysosomal enzymes released by neutrophils and macrophages

Answer 517

responsible for phagolysosome formation, inducible Nitric oxide synthase release, and the development of granulomas and caseous necrosis

Answer 518

osteoblastic: bone-specific Alk phos blasts synthesize the bone matrix, releasing Alk Phos in the process. Osteoclastic: urinary deoxypyridinoline urinary hydroxyproline TRAP

Answer 519

inherited myocardial repolarization defect, commonly: mutated K channel, giving you a delayed rectifier current (Ik) of the cardiac AP 2 syndromes: Romano-Ward syndrome (prue cardiac) Jervell and Lange-Nielsen (cardiac + deafness)

Answer 520

white/yellow plaques on colonic mucosa, indicating pseudomembranous colitis

Answer 521

gastronomas in SI/pancreas cause ulcers, heartburn, and diarrhea elevated gastrin levels that RISE in response to secretin. secretin normally decreases gastrin from normal G cells.

Answer 522

PRPP aminotransferase this is the enzyme in the de novo purine synthesis pathway, because Lesch Nyhan takes out HGPRT, which is required for the purine salvage pathway.

Answer 523

intracranial HTN (papilledema) dry skin, alopecia HSM teratogenic!

Answer 524

Amatoxins are found in mushrooms, and are potent inhibitors of RNA polymerase 2, which halts mRNA synthesis

Answer 525

myoglobin is monomeric so it does not show positive cooperatively and is not sigmoid. it just jumps up immediately. the individual subunits of Hb molecule are structurally analogous to myoglobin, so you if you isolate them they'll look like myoglobin on a curve

Answer 526

Hawthorne: observer effect; subjects change behavior once they know they're being studied Pygmalion effect: researchers' beliefs of the efficacy of a tx can affect the outcome- self fulfilling prophecy Berkson bias: selection bias from choosing hospitalized pts as the control (when they're less healthy than the general population)

Answer 527

it's used in case-control to try to help control confounding matching variables should always be the potential confounders (age, race, etc). Cases and controls are then selected based on matching variables so that both groups have similar distribution in accordance with these variables

Answer 528

glycogen breakdown is synchronized with skeletal muscle contraction via release of sarcoplasmic Calcium after NT stimulation. increased intracellular Ca causes activation of phosphorylase kinase, which stimulates glycogen phosphorylase to increase glycogenolysis cAMP stimulates glycogen breakdown, but it is not responsible for synchronization

Answer 529

HbA2 alpha2delta2

Answer 530

HbH alpha thalassemia intermedia beta4 HbBarts alpha thalassemia major gamma4 incompatible with life

Answer 531

Gq activates phospholipase C, then membrane proteins are broken down into DAG And IP3. Protein Kinase C is then activated by DAG and calcium Calcium is released from the ER under the influence of IP3

Answer 532

the majority of the TCA cycle generates NADH and FADH2, but GTP is also produced in the TCA cycle: Succinyl-CoA to Succinate via succinyl-Coa GTP is used during gluconeogenesis to synthesize phosphoenolpyruvate (PEP) from Oxaloacetate (with PEP carboxylase)

Answer 533

t-test: differences between means of 2 groups "t is MEANt for 2" ANOVA: Analysis of Variance 3 words, so checks differences between means of 3 or more groups Chi square: checks differences between categorical outcomes (not mean values) "chi-tegorical"

Answer 534

temporal: impulses from the same neuron spatial: summation of impulses from several different neurons

Answer 535

Burkitt Lymphoma: t(8;14) c-myc activation "8urkitt's lymphoma" ``` CML: t(9;22) BCR-ABL Philadelphia Chromosome "9hiladelphia CreaML cheese" ``` ``` Mantle Cell lymphoma t(11;14) CKD1 activation "Mantle ce11 lymphoma" "11's are candles on a mantle" ``` Follicular lymphoma t(14;18) BCL2 Activation "4-1cular lymphoma" Ewing Sarcoma: t(11;22) fusion protein EWS-FL1 "A plane is twice as long as its (e)wing; just like 22 is twice as much as 11" ``` APL (AML M3 type) t(15;17) Auer rods tx w/ ATRA "All trans pPL start playing with their "rods" by 15" ```

Answer 536

NF-kB is a transcription factor normally latent in the cytoplasm because it is bound to its inhibitor protein, IkB. removing IkB activates NF-kB. NF-kB plays a critical role in immune response to infection and inflammation. Active NF-kB promotes nuclear transcription of inflammatory proteins like cytokines, Acute phase reactants, cell adhesion molecules, and leukocyte-related growth factors. glucocorticoids activate IkB to keep NF-kB latent

Answer 537

resp droplets human-human gravity-assisted entry of small, TB-laden droplets to lower lung fields phagocytized by alveolar macrophages sulfatide virulence factor allows for intracellular proliferation macrophage lysis/release more macrophage invasion, recruitment, infection eventually, antigen-carrying macrophages or DC's go to lymph generate helper T response (2-4 weeks) T cells secrete IFN for macrophage activation, which allows them to form epithelioid cells improves ability to kill M tuberculosis

Answer 538

coordinate contraction of all myofibrils they're invaginations of the sarcolemma that extend into each muscle fiber. they transmit depo signals to the SR and trigger release of Ca

Answer 539

spirochete!!! (spiral galaxy) Treponema Pallidum tertiary presents with: Tabes dorsalis Argyll Roberston pupils

Answer 540

it's a sheep tapeworm that forms hydatid cysts with "eggshell" calcifications in the liver. it can cause anaphylaxis if you release the antigens during surgery,so ethanol is used to kill the daughter cysts first

Answer 541

pheo: hypertension 2/2 catecholamine release carcinoid: hypotension, facial flushing, bronchospasm, diarrhea, usually 2/2 neuroendocrine Serotonin tumor

Answer 542

preterm, formula fed infant. necrosis of intestine mucosa with possible perforation, which can lead to pneumatosis intestinalis (parallel air in the bowel wall) free air in abdomen, and portsl venous gas

Answer 543

``` widespread MSK pain fatigue cognitive impairment ("fibro fog") multiple soft tissue tender points absent inflammation normal lab findings ```

Answer 544

product of lipid peroxidation it is tinged yellow-brown and accumulates in normal aging cells, but especially in pts with malnutrition and cachexia. commonly seen in liver and heart

Answer 545

bilateral wedge-shaped strips of necrosis over the cerebral convexity, parallel and adjacent to the longitudinal cerebral fissure these are watershed infarcts between the zones of perfusion of the ACA, MCA, and PCAs

Answer 546

initiation: ischemic injury to renal tubules maintenance stage: oliguria, fluidoverload, increasing creatine/BUN, metabolic acidosis, hyperkalemia (pts have edema and decreased excretion and retain H+) recovery: polyuria, BUN and Cr fall; risk of hypokalemia (pts become dehydrated w/ high vol, hypotonic urine)

Answer 547

pulmonary vascular resistance is lowest at functional residual capacity increased lung vol's increase PVR due to longitudinal stretching (wall to wall) of alveolar capillaries by the expanding alveoli. decreased lung vol's also increase PVR due to decreased radial traction from adjacent tissues on the large extra-alveolar vessels

Answer 548

child: erythematous infection (fifth disease) adult: symmetric polyarthritis it can mimic Rheumatoid Arthritis but it's usually self-resolving

Answer 549

both associated with chronic HTN and the same small penetrating arterioles as lacunar stroke Charcot Bouchard aneurysm: rupture leads to intraparenchymal hemorrhage in the deep structures, that would show up on CT Hypertensive arteriolar sclerosis: hardening/thickening of the vessel wall that predisposes it to very small infarct or occlusion, which does not show up on CT

Answer 550

posterior limb of the INTERNAL CAPSULE or basal pons lesion, like an intraparenchymal hemorrhage