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USMLE Step 1 > Stuff You Missed II > Flashcards

Stuff You Missed II Flashcards

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1
Q

Three important metabolic pathways for glucose

A
  1. Glycolysis and TCA cycle - used to generate ATP. Glucose is first metabolized to pyruvate during glycolysis and then pyruvate is converted to acetyl-COA, which enters TCA cycle
  2. Glycogenesis stores glucose for later use avia formation of glyogen from GLUCOSE-1 PHOSPHATE
  3. HMP shunt (pentose pathway) generate pentose sugars and NADPH
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2
Q

Oxidative portion of HMP shunt

A
  • Is irreversible
  • Rate limiting enzyme: Glucose 6 phosphate dehydrogenase (G6PD)

GLUCOSE-6-PHOSPHATE is converted to RIBULOSE-5-PHOSPHATE which results in NAPDH formation.

-

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3
Q

Non-oxidative portion of HMP shunt

A
  • Is reversible
  • key enzyme is TRANSKETOLASE (B1 dependent)
  • Used to convert sugars to ribose-5-phosphate (for nucleotide synthesis) and intermediates (fructose 6 phosphate and glyceraldehyde-3-phosphate) for glycolysis and gluconeogeneis

-

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4
Q

Discuss NADPH and Glutathione

A

NADPH (made in oxidative part of HMP shunt) reduces glutathione, a co-enzyme for gluthathione peroxidase which prevents oxidative damage by converting H2O2 into H2O

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5
Q

Where is the oxidative phase of HMP shunt most active?

A

In tissues that use NADPH in reductive pathways

  • Adipose tissue for fatty acid sythesis
  • Adrenals for steroid synthesis
  • Glutathione reduction in RBC
  • Liver for fatty acid and steroid synthesis
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6
Q

Glucose 6 Phosphate Dehydrogenase Deficiency

A
  • X-linked recessive disease with predominance in Asia, Mediterranean, Africa (malaria resistance countries)
    Hemolytic anemia when RBCs are exposed to oxidative stress because they lack sufficient NADPH production thus less glutathione activity
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7
Q

Causes of oxidative stress

A

Can be dangerous in case of glucose 6 phosphate dehydrogenase

  • Infections
  • Fava beans (“broad beans”)
  • Drugs (e.g. sulfonamides, dapsone, primaquine)
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8
Q

Describe peripheral smear of patient with Glucose 6 Phosphate Dehydrogenase

A

RBCs have Heinz bodies (intracellular inclusions composed of denatured hemoglobin)

  • Also contain degmacytes (bite cells) - result of splenic macrophages removing Heinz Bodies
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9
Q

Hepatocellular Carcinoma - Presentation

A

Liver mass with increased alpha fetoprotein

  • associated with HBV
  • integration of viral DNA into host cellular genome is trigger for neoplastic changes
  • HBx activates IGF-2 and IGF-1 to stimulate cell proliferatin
  • HBV also suppresses p53 tumr suppressor, leading to accumulations of mutations in hepatocytes leading to carcinogenesis
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10
Q

Risk factors for Hepatocellular Carcinoma

A
  • HBV and HCV
  • Alcoholic Cirrhosis
  • Alfatoxins
  • Hemochromatosis
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11
Q

Anal fissure

A

tear in lining of canal DISTAL to dentate line that occurs most often on posterior midline.

  • Patients complain of severe tearing pain associated with passage of bowel movements
  • Associated with low-fiber diets and constipation
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12
Q

Parietal cells

A

release H+ ions into gastric lumen via H/K ATPase , which requires ATP hydrolysis thus active transport mechanism

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13
Q

Omeprazole

A

suppress activity of gastric parietal cell H/K/ATPase leading to an increase in pH of gastric lumen

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14
Q

Lidocain

A

Local anesthetic
- Class IB antiarrhythmic agen that blocks voltage gated Na channels in sensory neurons, Purkinje fibers, and ventricular cells

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15
Q

Dofetilide

A

Class III (K+ blocker) that blocks passive transport of potassium, in cardiomycocytes leading to prolongation of refractory period and QT interval

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16
Q

Pancreatic divism

A

incomplete fusion of ventral and dorsal pancreatic buds. Usually asymptomatic, but may predispose to acute or recurrent pancreatitis.

  • No duodenal obstruction
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17
Q

Annular pancreas

A

Due to abnormal migration of ventral pancreatic bud which encircule the duodenum and may lead to sx of duodenal obstruction in neonates

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18
Q

Zenker’s diverticulum

A
  • due to cricopharyngeal muscle dysfunction caused by diminished relaxation of pharyngeal muscles during swallowing
  • Found in upper esophagus
  • classically older patients presenting with oropharyngeal dysplasia, coughing, choking, and recurrent aspiration
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19
Q

True diverticula (of esophagus)

A

Due to scarring and traction of esophagus.

- Seen in midportion of esophagus and result from mediastinal lymphadenitis (TB, fungal infections)

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20
Q

Intussusception

A

occurs in children younger than 2 and in region of ileocecal valve
- presents with intermitten, severe, colicky abdominal pain, “currant jelly” still and possible palpable mass in RUQ

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21
Q

Fever, jaundice, anorexia in IV drug user

A

Suggests viral hepatitis (likely caused by Hep C)

- acute viral hepatitis causes hepatocyte apoptosis and necrosis

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22
Q

Effects of viral hepatitis on hepatocytes

A
  • Apoptotic hepatocytes shrink, undergo nuclear fragmentation and become intensely eosinophilic
  • Eosinophilic bodies called Acidophillic bodies, Councilman bodies, and apoptotic bodies
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23
Q

Hep A presentation in children

A

Commonly silent or subclinical (“anicteric”) in children but can present as an acute ilnness characterized by jaundice, malaise, nausea, vomiting, and RUQ pain

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24
Q

Thiamine (B1) is a co-factor for which 3 important enzymes?

A
  1. Transketolase (in nonoxidative HMP shunt)
  2. Alpha ketoglutarate Dehydrogenase (Alpha ketoglutarae to Succinyl-CoA) in TCA cycle
  3. Pyruvate Dehydrogenase (Pyruvate –> Acetyl-CoA)
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25
Q

Co-factors needed for Alpha Ketoglutarate Dehydrogenase

A
  1. Thiamine
  2. Lipoic Acid
  3. CoA
  4. FAD
  5. NAD
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26
Q

Areas most susceptible to ischemia with ischemic colitis

A
  1. SPLENIC FLEXURE (lies between SMA and IMA)

2. DISTAL SIGMOID COLON (lies between IMA and hypogastric arteries)

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27
Q

Salmonella Typhi

A
  • causes typhoid or enteric fever
  • faculative intracellular parasite orally, penetrates intestinal mucosa and travels to mesenteric lymph nodes where it is phagocytosed by macrophages for survival
  • characterized by fever, abdominal pain, diarrhe, and “rose spots”
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28
Q

Pseudomonas Aeruginosa

A
  • produces enterotoxin A, similar to diptheria toxin that halts human cellular protein synthesis by inhibiting EF-2
  • Arrest in translation as protein chain is unable to change positions in ribosome
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29
Q

Vibrio cholerae

A
  • causative agent of cholera and produces exterotoxin, CHOLERAGEN
  • Choleragen acts like heat-labile toxin of ETEC in that increases cAMP in intestinal mucosal cells leading to decreased absorption and increased secretion of Na, Cl, and water
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30
Q

C. difficile

A
  • produces toxin A (enterotoxin) and toxin B(cytotoxin) and causes pseudomembranous colitis
  • mostly seen in patients recently treated with antibiotics
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31
Q

Toxin B of C. difficile

A

Depolymerizes actin filaments causing GI mucosal cells death

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32
Q

Enterohemorrhagic E. coli (EHEC) toxin

A
  • produces Shiga-like toxins (aka Vero cytotoxins)
  • identical to Shiga toxin produced by Shigella dysenteriae
  • toxins inhibit 60S ribosomal subunits thus blocking protein synthesis by preventing binding of tRNA
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33
Q

Right sided colon cancer vs. Left sided colon cancer

A

Left sided colon cancers manifest as partial intestinal obstruction (because right sided colon has larger lumen)
Right sided colon cancers present with symptoms of iron deficiency and systemic symptoms

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34
Q

Gastroduodenal artery

A

Lies along posterior of duodenal bulb (1st part of duodenum). It is more likely to be eroded by posterior duodenal ulcers.
- Ulceration into the gastroduodenal artery is source of life-threatening hemorrhage

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35
Q

Brown pigment gallstones

A
  • Arise secondary to infection of billiary tract which in turn releases B-glucuronidase by injured hepatocytes and bacteria
  • B-glucoronidase hydrolyzes bilirubin glucuronides and increases amt of uncojugated bilirubin in bile
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36
Q

Infections associated with brown pigment gallstones

A

E. coli
Ascaris lumbricoides
Liver Fluke (Opisthorichis sinesis)

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37
Q

Populations associated with brown pigment stones

A
  • Rural Asian populations
  • Women
  • Elderly
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38
Q

7-alpha hydroxylase

A

Converts cholesterol to bile acids. Deficiency leads to cholesterol gallstone

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39
Q

First order kinetics (of Pharmocology)

A

Same PROPORTION of drug is metabolized per unit of time - thus linked to drug concentration

(e.g. For 20 mg dose of Drug X, 10mg will be metabolized in hour. For 40 mg of Drug X, 20 mg will be metabolized in 1 hr)

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40
Q

Zero order kinetics (of Pharmacology)

A

Same AMOUNT of drug is metabolized independent of drug dose

  • If 30 mg of drug Y was administered, 10 mg will be metabolized in one hour.
  • If 60 mg of drug Y was administered, 10mg will be metabolized
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41
Q

What slows progression of hemochromatosis in women?

A

Physiological iron loss through menstruation and pregnancy

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42
Q

Sx of hemachromatosis

A
  • Micronodular cirrhosis
  • Diabetes mellitus
  • Skin pigmenation
  • Cardiac dysfunction due to accumulation of Fe in myocardium
  • Hepatomegaly
  • Cardiomegaly
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43
Q

CREST

A 
C-alcinosis
R-aynaud Syndrome
E-sophageal dysmotility
S-clerodactly
T-elegienctasia
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44
Q

Early sign of systemic sclerosis

A

Esophageal dysmotility and incompetence of lower esophageal sphincter

  • Result of atrophy and fibrous replacement of esophageal muscles
  • Esophageal dilation causes reflux, increases risk of Barrett’s esophageus and esophageal adenocarcinoma
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45
Q

C. difficile toxins

A

Toxin A - attracts neutrophils to cause mucosal inflammation, loss of water in the gut, and diarrhea
Toxin B - causes actin depolymerization, loss of cytoskeleton integrity, cell death, and mucosal necrosis

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46
Q

Colon adenocarcinoma

A
  • Rectosigmoid colon - most common location

- Often present with obstructing glands

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47
Q

Colon adenocarcinoma: Pathology

A

Consist of pleomorphic cells with large dark nuclei forming irregular crowded glands, some of which contain mucus

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48
Q

Gallstone ileus

A

results from passage of large gallstone (>2.5 cm) throug cholecystenteric fistula into small bowel causing obstructing to ileocecal valve.

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49
Q

Gas within gallbladder and biliary tree on abdominal X-ray suggests what?

A

Presence of cholecystenteric fistula to small bowel as intestinal gas can now enter gallbladder.
-Predisposes gallstone ileus

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50
Q

TPN-induced gallstone

A
  1. Biliary stasis from absent enteral stimulation due to decreased CCK release
  2. In those with ileal resection, disturbance of bile acid circulation results in supersaturation of hepatic bile with cholesterol
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51
Q

Antibodies directed against HCV envelope proteins don’t confer immunity because?

A

HCV envelope proteins vary their antigenic structure, thus production of host antibodies always lag behind the mutant strains and thus immunity is not given

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52
Q

Sx of adult lead poisoning

A
  1. Colicky abdominal pain, constipation, impaired concentration
  2. Bluish pigmentation at gum-line
  3. Wrist drop due to peipheral neuropathy
  4. MICROCYTIC Anemia and Basophilic stippling
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53
Q

Stages of Lead Poisoning (Hint: 4)

A
  1. Nausea, diarrhea, and abdominal pain. Hemorrhage, Hypovolemia, and Shock.
  2. GI symptoms resolve and patient appears better
  3. Metabolic acidosis, Hepatitic dysfunction, and Hypoglycemia
  4. Scarring of recovering GI tract
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54
Q

Cholestasis can cause malabsorption of which vitamins?

A

Fat soluble vitamins (e.g. A,D,E, K)

  • Osteomalacia may result of poor Vitamin D absorption
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55
Q

Cholestasis

A
  • can arise secondary to hepatocellular dysfuncton and intrahepatic or extrahepatic biliary obstruction
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56
Q

Esophagus (on CT)

A

Found between vertebral bodies in upper thorax and the trachea (remains open). It is typically collapsed and invisible on CT.

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57
Q

Are gastric ulcers from peptic ulcer disease seen on distal duodenum?

A

No. They are typically found on antrum, pyloric area of stomach or in first part of duodenum

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58
Q

Zollinger- Eilison Syndrome

A

Occurs to hypersecretion of gastrin by cardiac tumor.

  • Gastrin increases gastric acid production whcih causes peptic ulcers.
  • Diarrhea is common symptom in Z-E as excess gastric acid inactivates pancreatic enzymes
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59
Q

Pantothenic Acid (Vitamin B5)

A
  • Needed form coenzyme A, essential for aceylation reactions

- Binds with oxaloacetate in first step of TCA to form citate and then succinyl-CoA

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60
Q

Sx of Carcinoid syndrome

A
  • Flushing
  • Asthma-like symptoms (wheezing, dyspnea)
  • Diarrhea
  • Syncope (low blood pressure)
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61
Q

Carcinoid syndrome

A
  • Sign of GI metastesis
  • Associated with appendiceal tumors which secrete active substances like serotonin
  • If tumor is localized to GI tract, liver clears serotonin before any systemic symptoms can appear
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62
Q

Ondansetron

A

potent anti-emetic mainly used in patients undergoing chemotherapy. Selectively blocks 5-HT3 receptors.

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63
Q

Treatment of Carcinoid Syndrome

A

Octeotride - somatostatin analog that can inhibit secretion of many hormones and horomone-like substances

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64
Q

Secretin

A
  • produced S cells in the DUODENUM

- stimulates Bicarb production in pancreas

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65
Q

Secretin release stimulated by what?

A

Excess HCl

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66
Q

Hepatocyte injury in viral hepatitis

A
  • Diffuse swelling termed “balloow dengerating”
    Hepatocyte death characterized by lobular architecture disruption and hepatocyte necrosis called “bridging fibrosis
    -Presence of mononuclear inflammation
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67
Q

Common side effects for nitrates

A

Headaches and cutaneous flushing due to vasodilation caused by nitrates (including nitroglycerin and isosorbide)

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68
Q

Common cause of mitral regurgitation in undeveloped countries

A

Rheumatic fever

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69
Q

Strep viridans and adherence during subacute endocarditis

A

S. viridans - normal oral cavity inhabitans

  • Dextrans produced by S. viridans with sucrose as substrate facilitate bacterial adherence and colonization to fibrin
  • MUST HAVE PRE-EXISTING DAMAGE
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70
Q

S. aureus vs. Strep viridans in endocarditis

A

S. aureus can adhere to INTACT/ NON-DAMAGED endothelium to cause endocarditis

S. viridans can only attach to ALREADY DAMAGED valvular leaflet to cause endocarditis

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71
Q

Genetic mutation in Hypertrophic cardiomyopathy

A

Due to defect in cardiac sarcomere proteins. Most common defect is is Beta-myosin heavy chain

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72
Q

Dilated cardiomyopathy

A

Majority cases are due to autosomal dominant mutaitons of cardiac myocyte cytoskeletal proteisns (dystrophin) or mitochrondrial enzymes

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73
Q

Sx: of coarctation of aorta

A

Diminished femoral pulses compared to brachial pulses, symptoms of inadequate perfusion of lower extremities

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74
Q

Genetic syndrome associated with Coarctation of Aorta

A

Turner’s Syndrome

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75
Q

Cardiac defect in Down Syndrome

A

Endocardial Cushion Defects (Ostium primum ASD, regurgitant atrioventricular valves)

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76
Q

Cardiac defect in DiGeorge Syndrome

A

Tetralogy of Fallot and interrupted aortic arch (truncus arteriosus)

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77
Q

Cardiac defect in Friedrich’s ataxia

A

Hypetrophic cardiomyopathy

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78
Q

Cardiac defect in Marfan’s Syndrome

A

Cystic medial necrosis of the aorta

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79
Q

Cardiac defect in Tuberous Scleross

A

Valvular obstruction due to cardiac rhabdomyoma

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80
Q

Most likely cause of fatigue and new-onset murmur in young adult

A

Bacterial endocarditis. Acute, diffuse, proliferative glomerulonephritis secondary to circulating immune complexes may complicate BE and result in renal insufficiency

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81
Q

Long term hemodynamic compensatory response to volume overload of Aortic Regurgitation

A

Increase in left ventricular preload (LV end-diastolic volume) in associated with ECCENTRIC left venticular hypertrophy

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82
Q

Concentric hypertrophy of the LV

A

Response to pressure overload, involves in PARALLEL deposition of sarcomeres which results in wall thickening and decreased EDV

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83
Q

Cyanotic neonate with severe tachypnea and continuous machine like murmur
ECG shows aorta that lies anterior to and to the right of pulmonary artery

A

Transposition of Great Arteries

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84
Q

Transposition of Great Arteries

A

Failure of aorticopulmonary septum to spiral normally during fetal development.

  • Result is aorta is connected to right ventricle and pulmonary artery is connected to left ventricle
  • Only VSD, PDA, or ASD allows this to be compatible with lidfe
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85
Q

Number Needed To Treat

A

1/ Absolute Risk Reduction

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86
Q

Absolute Risk Reduction

A

(No disease/ Total receiving placebo) - (No disease/ Total receiving treatment)

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87
Q

Sx: Atrial Myxoma

A
  • Constitutional symptoms
  • Mid-diastolic rumbling murmur heard at apex (mitral stenosis)
  • Large penduculated mass in LA
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88
Q

Atrial Myxoma

A

Tumor compoed of scattered cells with mucopolysaccharide stroma, abnormal blood vessels, and hemorrhaging

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89
Q

Patient presents with hx of malabsorption complains of pruritic skin rash over extensor surfaces of arms.

A

Dermatitis herpetiformis

  • associated with celiac disease
  • erythematous, puritic papules, vesicles, bullae that appear over extensor surfaces
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90
Q

Treatment for acute gouty arthritis

A

NSAIDs, Colchicine

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91
Q

Colchicine

A
  • affects tubulin polymerization into microtubules which disrupts chemotaxis and phagocytosis
  • associated side effects: nausea, abdominal pain, and diarrhea
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92
Q

Caudal regression syndrome

A

characterized by sacral agenesis causing lower extremity paralysis and urinary incontinence
- associated with poorly controlled maternal diabetes

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93
Q

Cocaine abuse on fetus

A
  • Due to vasocontrictive effect, can cause insufficient blood flow to fetus, results in spontaneous abortion, intrauterine growth retardation, placental abruption, and/or prematurity
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94
Q

Congenital effect of rubella

A
  • Can cause mental retardation and congenital heart defects
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95
Q

Congenital effects of Vitamin A overdose

A

Craniofacial abnormalities
Posterior fossa CNS defects (Arnold-Chiari syndrome??)
- Auditory defects
- Abnormalities of great vessels (similar to DiGeorge)

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96
Q

Iron deficiency during pregnancy

A

Can restrict fetal growth

- important in 2nd half of pregnancy when maternal blood volume expands a lot

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97
Q

PTH effects on calcium and phosphate

A
  • increases serum calcium level
  • mediates bone resorption by causing osteoBLasts to inc. production of RANK-ligant and M-CSF to stimulate osteoCLasts
  • decreases serum phosphate (loss in urine)
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98
Q

Manifestations of sickle cell disease

A
  1. HEMOLYSIS - repeated sickling of RBCs to leads to permanent sickling and premature erythrocyte destruction
  2. VASOOCCLUSIVE symptoms: pain from small bone or bone marrow infarctions in hand, feet, wrist, and ankles (dactylitis)
  3. INFECTIONS - predisposed to encapsulated organisms due to repeated splenic infarcts
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99
Q

Dactylitis

A
  • Painful swelling of hands and feet

- common vasooclusive presentation of sickle cell anemia in young children

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100
Q

Hemolysis and haptoglobin levels

A

In hemolysis, haptoglobin levels decrease by binding to free hemoglobin and the complex is hepatically cleared

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101
Q

Raloxifene

A

Selective Estrogen Receptor Modulator (SERM)

  • binds to estrogen receptors and exhibits tissue-specific behavior that antagonize natural estrogen effects
  • IN BONE, acts like estrogen AGONIST and inhibits osteopeross
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102
Q

Effects of raloxifene in breast vs. bone

A

In breast, raloxifene is an estrogen ANTAGONIST thus useful treatment for estrogen-positive breast cancer

In bone, raloxifene is an estrogen AGONIST and inhibits osteoporosis

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103
Q

Markers for osteoblast activity

A

Bone-specific osteoblastic activity

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104
Q

Markers for osteoclast activity

A
  • Tartrate-resistant acid phosphatase
  • Urinary hydroxyproline
  • Urinary deoxypyridinoline (most reliable)
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105
Q

Vitamin D analogs and Psoriasis

A

Vitamin D analogs (calcipotriene, calcitrol, and tacalcitol) are used to treat psoriasis

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106
Q

Calcipotriene, Calcitriol, Tacalcitol

A
  • Vitamin D analogs, can be used to treat psoriasis

- bind to Vitamin D receptor (bind and inhibit KERATINOCYTE PROLIFERATION and stimulate KERATINOCYTE differentiation

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107
Q

Ustekinumab (Stelara)

A

human monoclonal antibody used to treat psoriasis by targeting IL-12 ad IL-23
- Inhibits differentiation of CD4+ Th1 and Th17 cells

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108
Q

Cyclosporine

A
  • inhibits NFAT (nuclear factor of activated T cells) from entering nucleus and modulating transcription activity by impairing production and relaese of IL-2 thus inhibiting T-cell proliferation
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109
Q

Keloid formation

A
  • result from excessive collagen formation
  • present as soft/firm nodules BEYOND borders of wound with “claw-like” extensions into normal tissue
  • composed of large disorganized collagen bundles and excess cellular connective tissue
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110
Q

Most likely diagnosis

- young athlete in 20s with nodular and comedonal acne

A
  • Likely from anabolic steroid (e.g. methyltestosterone) use. Androgens stimulate epidermal follcular epidermal hyperproliferation and excessive sebum leading to acne
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111
Q

Elements of pathophysiology of acne (hint: 4)

A
  1. Follicular epidermal hyperproliferation
  2. Excessive sebum production
  3. Inflammation
  4. Propionibacterium
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112
Q

Reiter’s syndrome (reactive arthritis) triad

A
  1. Urethritis
  2. Conjunctivitis
  3. Arthritis
    - associated with HLA-B27
    - seen weeks after a GU or GI infection
    - may be seen with sacroillitis
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113
Q

Lentigos

A

-small brown/tan macules seen in sun exposed skin of elderly person

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114
Q

RBC cell extravasations

A

Petechiae (< 5mm)
Purpurae (5mm to 1cm)
Echymoses (> 1 cm)

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115
Q

Telangiectasias

A
  • small permanent dilations of superficial capillaries and venules
  • usually found on skin or mucous membranes
  • blanch under pressure
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116
Q

Achondroplasia

A
  • autosomal dominant disease due to mutation of FGFR-3
  • patients present with normal spine length but short arms and legs with normal sized head
  • issue with endochrondrall ossification (long bones) not membranous ossification (flat bones)
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117
Q

Supraspinatous muscle

A
  • assists in abduction of arm and stablization of glenohumeral joint
  • tendon is commonly injured of rotator cuff muscles
  • commonly injured due to impingement of acromion and head of humerus`
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118
Q

Infections associated with reactive arthritis

A 
Chlamydia
Campylobacter
Salmonella
Shigella
Yersinia
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119
Q

Skeletal muscle contraction

A
  • Ca from SR binds to TROPONIN C to uncover binding sites on actin
  • Myosin (energized by ATP+Pi) binds to actin
  • Once myosin is bound, Pi is released which changes myosin head conformation and causes power stroke
  • After power stroke, ATP binds myosin head causing myosin detachment from actin
  • ATP hydrolysis (ATP –> ADP+Pi) energizes myosin for next contraction
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120
Q

Smooth muscle contraction

A
  • Ca (Sarcoplasmic Reticulum OR ECF) binds to calmodulin to activate myosin-light chain kinase
  • Phosphorylated MLCK more likely to bind with actin
  • *Unphosphorylated MLCK has low affinity for actin. Actin is NOT covered up)
  • As long as Ca is present, MLCK will stay bound to actin
  • Ca-ATPase will remove Ca once stimulus is over
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121
Q

Treatment for male pattern baldness

A

Finasteride (5-alpha-reductase) inhibitor because male pattern baldness involves 5-alpha reductase and androgenic effects of dihydrotestosterone

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122
Q

Mitochondrial myopathy

A

Patients present with Neuromuscular lesions
Ragged red skeletal muscle fibers, and
Lactic acidosis
- Mitochondrial disorder thus follows maternal inheritence

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123
Q

Why do mitochondrial defects cause lactic acidosis?

A

Mitochondria responsible for OXPHOS.

No mitochondria = No OXPHOS thus presence of lactic acidosis

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124
Q

Heteroplasmy

A

Variable clinical expression of mitochondrial DNA defects in different family members due to co-existence of mutated and wild type mitochondrial genomes in each cell

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125
Q

Possible structures damaged during injury to mid-shaft of humerus

A

Radial nerve
Deep brachial artery

** Supracondylar fractures associated with injury to brachial artery

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126
Q

Physical findings of rickt

A
  • Unmineralized osteoid matrix
  • Widened osteoid seams
  • Bowed legs
  • Rachitic rosary (prominence at costevertebral junction)
  • Harrison’s sulci (indentations in lower ribs)
  • Craniotabes
  • Growth retardation
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127
Q

Paget’s disease of bone findings

A
  • mosaic of lamellar bone, irregular sections of lamellar linked by “cement lines”
  • local osteoclastic activity followed by increased osteoblastic activity
  • formation of abnormal bone
  • new collagen is laid haphazardly
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128
Q

Common fracture sites in osteoporosis

A
  • Primarily involves trabecular bone
  • patients might present with fractures in lumbar vertebrae (mostly trabecular) or in neck of femur (50% trabecular/50% cortical)
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129
Q

Osteoporosis

A

represents loss of “total bone mass” that results in trabecular thinning.
-common fracture sites are: lumbar vertebrae and neck of femur

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130
Q

Leprosy

A
  • caused by Mycobacterium lepare
  • deforming infection of skin and nerves
  • associated with armadillo contact in SW U.S.
  • severity depends on strength of cell-mediated immune response, with tuberculoid leprosy and lepromatous leprosy (more severe form)
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131
Q

African patient presents with patchy areas of skin anesthesia and hypopigmentation in upper extremities. Microscopy shows bacteria invading Schwann cells. Likely diagnosis?

A

Leprosy (Hansen’s disease)

- caused by Mycobacterium leprae

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132
Q

Measles virus

A

enveloped, non-segmented, negative sense RNA virus

causes Rubeola

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133
Q

Measles (Rubeola)

A
  • caused by measles virus (paramyxovirus)
  • Kolik spots (white/blue-gray lesions on buccal mucosa that precede maculopapular rash
  • Cough
  • Coryza
  • Conjunctivitis
  • vaccine given at 12-15 months
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134
Q

Mneumonic for Measles Diagnosis

A 
"CCCK"
C-oryza
C-ough
C-onjunctivitis
K-olik spots (white/blue-gray spots on buccal mucosa)
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135
Q

Rotavirus symptoms

A

Upper respiratory infection followed by diarrhea

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136
Q

Xanthelesma

A
  • eyelid papule or plaque containing lipid-laden macrophages
  • occur in primary or secondary hyperlipidemia
  • Biliary cirrhosis is potential cause of hyperlipidemia
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137
Q

Cutaneous manifestations of HYPERthyroidism

A
  • Thinning hair
  • Pretibial myxedema
  • Alopecia areata
  • Hyperhidrosis
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138
Q

Side effects of hydrochlorothiazide

A

HyperGLUC

HyperGlycemia
HyperLipidemia
HyperUricemia
HyperCalcemia

** can be used for women who have osteoporosis and hypertension

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139
Q

Wound contractures

A
  • Due to excessive metalloproteinase activity and myofibroblat accumulation in wound margins
  • Contracture produce wound deformities and surrounding tissues esp in palms, soles, anterior thorax, or serious burn sites
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140
Q

Erythema infectiosum (fifth disease)

A
  • caused by parvovirus B19 and is transmitted commonly via respiratory route
  • Sx include bright-red rash on cheeks (“slapped cheeks”) and lace-like rash on trunk and extremities
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141
Q

Hand and foot mouth disease

A
  • caused by Coxsackie virus type A
  • presents with dever and ulcers on tongue and oral mucosa
  • maculopapular and/or vesicular rash on palms and soles
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142
Q

Scarlet fever

A
  • caused by Group A Strep (Strep pyogenes)

- present with fever, sore throat, diffuse erythematous rash with small papules (“sandpaper-like”)

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143
Q

Ecthyma gangrenosum

A
  • cutaneous necrotic injury associated with Pseudomoans aeroginosa bacteremia and septicemia
  • P. aeruginosa invades perivascularly and releases exotoxins causing vascular destruction and resultant insufficiency of bloof to patches of skin which become edematous and necrose
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144
Q

Populations susceptible to P. aeruginosa

A
  • Neutropenic patients
  • Hospitalized patients
  • Patients with burns and chronic indwelling catheters
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145
Q

Dystonia

A
  • prolonged, repetitive muscle contractions
  • may be result of impaired function of ganglia
  • most common types of focal dystonia are cervical dystonia (spasmodic torticolis), blepharosparm, and writer’s crap
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146
Q

Myoclonus

A

sudden, brief sometimes serve (shock-like) muscle contraction
- associated with hiccups or hypnic jerks (when falling asleep)

Pathological myoclonus is seen in epilepsy and Creutfeldt-Jakob disease

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147
Q

Lepromin skin test

A

Tuberculoid patients will have positive test because they exhibit strong CD4+ Th1 cell-mediated immune response to M.leprae

  • Leprematous leprosy (more severe form) will have negative test because they exhibit weak immune response
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148
Q

Dantrolene

A
  • relax skeletal muscle by reducing release of Ca from SR

- used to treat malignant hyperthermia

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149
Q

Succinylcholine

A
  • depolarizing NMJ blocker administered during intubation and induction
  • can be augmented by neostigmine (anti-cholinesterase) during phase I block
  • can be reversed by neostigmine during phase II block
  • paralysis caused by succinylcholine depends on catabolism by plasma cholinesterase
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150
Q

Digital clubbing

A
  • “drumstick” sign at end of fingers
  • sign of prolonged hypoxia
  • often accompanies large lung-cell cancer, tuberculosis, cystic fibrosis, bronchiectasis and suppurative lung diseases (e.g empyema and chronic lung abcesses)
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151
Q

Androgenetic alopecia

A
  • most common cause of hair loss in both males and female

- demonstrates polygenic inheritance with variable penetrance`

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152
Q

Common condition affected by multiple genes

A
  • Androgenetic alopecia
  • Epilepsy
  • Ischemic heart disease
  • Schizophrenia
  • Glaucoma
  • Hypertension
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153
Q

Myasthenia gravis

A
  • autoimmune disease that results in decrease in acetylcholine receptors on post-synaptic terminal of muscle end plate
  • results in decreased ability of ACh to bind and open post-synaptic cation channels, thus decreasing end-plate potential
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154
Q

Treatment of myasthenia gravis

A
  • involves cholinesterase inhibitors, immunosuppresants, and somtimes thymectomy
  • cholinesterase inhibitors can be improved by antimuscarinics (e.g. scopolamine) to counter muscarinic overstimulation in sites like gut without affecting skeletal muscle function
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155
Q

Apocrine glands

A
  • found in breast areolae, axillae, genital regions of humans
  • secrete sweat into hair follicle instead of directly to skin surface
  • innervated by sympathetic fibers and not functional until puberty
  • secretions odorless until bacterial decomposition
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156
Q

Eccrine glands

A
  • also called merocrine glands
  • present in skin throughout most of body except areas like skin and glans pens
  • secrete water fluid rich in Na and Cl (think CF!!) directly to skin surface
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157
Q

Isoretinoin

A
  • treats severe acne refractory to topical therapies via interaction with RAR
  • main adverse effects: hypertriglyceridemia and teratogenicity
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158
Q

What test must be administered before giving isoretinoin to a young female?

A

Must give pregnancy test (test for B-hCG) because isoretinoin can cause birth defects?

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159
Q

Sx of Dermatomyositis

A
  • Gottron papules - flat topped plaques over bony prominences
  • Heliotrope rash - “racoon eyes”
  • Proximal muscle weakness, difficulty standing or rising up from chair
  • Anti-Jo 1 positive
  • elevated CK levels
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160
Q

Telomerase

A
  • enzyme that posesses reverse transcriptase (RNA-dependent DNA polymerase) activity and is normally expressed in stem cells as well as cancer cells
  • adds TTAGGG to 3’ end of DNA
  • Cancer cells continue to divide without aging and shortening telomeres
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161
Q

Gout

A

results from deposition of monosodium rate in joints and soft tissues
- underpolarized light, appear needle shaped and negatively birefringent

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162
Q

Psoriasis

A
  • salmon colored plaque covered with silvery scale
  • common areas include elbows, knees, gluteal cleft, lumbosacral area
  • may have yellow-brown discoloration on nails with associated pitting, thickening, or crumbling
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163
Q

Long thoracic nerve injury

A
  • commonly caused by mastectomy but any trauma or surgery in axillary area can damage this nerve
  • damage to this nerve causes “winged scapula” and inability to abduct shoulder past 90 degrees
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164
Q

Rheumatoid arthritis

A
  • swelling in pain, swelling, and morning stiffness for > 6 months
  • tends to get better as day progresses
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165
Q

Tx for rheumatoid arthritis

A

Short term therapy: glucocorticoids (quick and effective)

Long term therapy: Methotrexate (used for moderate to severe rheumatoid arthritis)

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166
Q

Hematogenous osteomyelitis

A
  • predominantly seen in children and affects long bones
  • caused by Staph. aureus
  • sx: include malaise, fevers, and pain over long bones
  • Bone pain caused by abscess within bone leading to periosteal disruption and bone necrosis
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167
Q

Moraxella cararrhalis

A
  • normal flora of upper respiratory tract of healthy adults and children
  • causes otitis media and sinusitis in healthy ppl
  • responsible for bronchitis and pneumonia in elderly patient with COPD
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168
Q

Sciatic nerve

A

L4 - S3

- splits into tibial nerve and common peroneal nerve

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169
Q

Tibial nerve

A
  • inverts and plantarflexes foot
  • when not working, the foot is everted and dorsiflexed (opposite of common peroneal nerve)

provides sensation to plantar aspect of foot

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170
Q

Most abundant amino acid in collagen

A

Glycine
- appears ever third amino acid position
as collagen formula is (-Gly-X-Y)333

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171
Q

Essential amino acid for formation of collagen alpha helix

A

Proli

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172
Q

Septic arthritis

A
  • usually due to gonococcus infection, common cause of infection in your adults
  • suspect if affected joint has high WBC count but absent crystals
  • ## requires immediate treatment (e.g. ceftriazone) to prevent joint destriction, osteomyelitis, and sepsis
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173
Q

Bacteroid species

A

Gram - anaerobic rods

  • areas of necrosis and diminished blood supply (e.g. chronic non-healing ulcers) facilitate growth of anaeroes
  • produce B-lactamase, which breaks down peniccilin
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174
Q

B-lactamase inhibitors

A

Tazobactam
Clavulanic acid
Sulbactasm

  • most effectie against most gram - enteric rods, including Pseudomonas aeruginoise and Bacteroides fragilis
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175
Q

Trauma or sustained pressure to neck of fibula causes injury to what nerve

A
  • Common peroneal nerve ( as it runs around neck of fibula)

- susceptible to traima from lateral blows of knee to tightly applied plaster casts

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176
Q

Migratory thrombophlebitis

A
  • should always think cancer
  • hypercoagulability is very common paraneoplastic syndrome seen in adenocarcinomas of pancrease, colon or lug
  • adenocarcinomas produce thromboplastin-like substance capable causing chronic intravascular coagulations
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177
Q

Cachexia

A

syndrome that encompasses anorexia, malaise, anemia, weight loss and generalized wasting due to underlying systemic disease.

TNF-alpha is a cytokine that causes necrosis of some tumors in utero
- Inhibitig

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177
Q

IVC

A

formed by union of right and left common illiac veins at level of L4-L5

  • Renal arteries lie at L1
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177
Q

Apoptosis

A

can occur either the intrinsic (mitochondria-mediated) pathway or extrinsic (receptor-initiated) pathway.
- Both pathways require activation of caspases that cleave cellular proteins

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177
Q

Foamy urine

A 
- caused by proteinuria/
Heavy proteinuria (e.g. nephrotic syndrome) can caused interstitial edema because of decrease in serum albumn
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178
Q

Migratory thrombophlebitis

A
  • should always think cancer
  • hypercoagulability is very common paraneoplastic syndrome seen in adenocarcinomas of pancrease, colon or lug
  • adenocarcinomas produce thromboplastin-like substance capable causing chronic intravascular coagulations
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180
Q

Auer rods

A

deformed azurophilic granules found in cytoplasm of myeloblasts that stain positively for MYELOPEROXIDASE

  • found in abundance in AML M3
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181
Q

Paroxysmal Nocturnal Hemoglobinuria

A

Triad: hemolytic anemia, hypercoaguable state, pancytopenia

  • causes thrombosis
  • deficiecy of CD55 and 59 which disallows complement activation
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181
Q

Migratory thrombophlebitis

A
  • should always think cancer
  • hypercoagulability is very common paraneoplastic syndrome seen in adenocarcinomas of pancrease, colon or lug
  • adenocarcinomas produce thromboplastin-like substance capable causing chronic intravascular coagulations
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182
Q

Migratory thrombophlebitis

A
  • should always think cancer
  • hypercoagulability is very common paraneoplastic syndrome seen in adenocarcinomas of pancrease, colon or lug
  • adenocarcinomas produce thromboplastin-like substance capable causing chronic intravascular coagulations
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184
Q

Hereditary spherocytosis

A

autosomal dominant condition that results form red blood cell cytoskeletaon abnormalities

  • most commonly spectrin and ankrin defects
  • definitive test: positive osmotic fragility test
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185
Q

Tx for hyperlipidemia

A

Niacin (Vitamin B3) - decreases synthesis of hepatic triglyceride and VLDL by suppressing release of FA from peripheral tissues

  • also decreases VLDL conversion to LDL, thus decreasing LDL
  • also increases HDL
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187
Q

Bile-acid binding resins

A

can cause constipation and abdominal bloating (potentiall worsening diverticulosis), hypertriglyceridemia, cholesteral gall stone and vitamin K malabsorption

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187
Q

Factor V Leiden mutation

A

causes factor Va resistance to inactivation by activated protein C, may account for approximately 20% of cases in atypcial venous thrombosis

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188
Q

Sx of hereditary spherocytosis

A

anemia, reticulocytosis and increased indirect bilirubin, indicating hemolytic anemia

  • MCHC (increased because of mild dehydration of red blood cell)
  • blood smear dysmorphic red cells with no central pallor
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189
Q

Henoch-Schonlein purpura

A
  • seen in young boys (3 - 10 years old)
  • palpable skin lesions associated with abdominal pain, arthralgias, and renal involvement
  • occurs due to deposition of Ig-A containing immune complexes and shows up in purpura on buttocks, legs
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190
Q

Glioblastoma multiforme

A
  • pallisading tumor cells around areas of necrosis
  • commonly crosses midline (“butterfly distribution”)
  • most common primary brain tumor in adults
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191
Q

AML (M3 variant)

A
  • associated with Auer rods
  • t(15;17) which codes for chimeric PML/RAR (retinoic acid receptor)
  • this inhibits differentiation of myeloblasts and triggers development of APML
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193
Q

Selective COX-2 inhibitors

A

e. g. celecoxib
- have no effect on platelet aggregation and cause little GI irritation
- the reduced risks of GI bleeding are only advantages over traditional NSAIDS

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194
Q

Microangiopathic hemolytic anemia

A
  • associaed with RBC fragments, Burr cells, and helmet cells

- in patients with prosthetic valves, RCs are exposed to excessive shear and turbulence in circulation causing damage

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194
Q

Glomangioma

A
  • very tender small red-blue lesion under nail bed

- originates from modified smooth muscle cells that control thermoregulatory functions of dermal glomus bodies

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195
Q

Sx: “Mismatched V/Q with perfusion defect, sudden onset chest pain

A

Signs of pulmonary embolism

- often caused by deep vein thrombosis and continuum of pulmonary thromboembolism

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197
Q

Bile acid-binding resins (cholestyramine, colestpiol, colesevelam)

A
  • bind to bile acid in GI tract, interfereing with its enterohepatic circulation
  • LDL is decreased b/c hepatic cholesterol is consumed in re-sythesis of bile acids
  • since bile is being re-synthesized, cholesterol content of bile increases thus increased risk of gallstone
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198
Q

Bcl-abl

A
  • associated with CML

- result of t(9;22) - Philadelphia chromosome

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199
Q

C-myc overexpression

A
  • associated with Burkitt’s lymphoma
  • associated with t(8;14)
  • myc oncogene on chr 8
  • Ig heavy chain on chr 14
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200
Q

Bcl-2 overexpression

A
  • associated with Follicular lymphoma
  • associated with t(14;18)
  • Bcl-2 has anti-apoptotic effects
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202
Q

Greater activity against Factor Xa and Thrombrin.

Unfractionated heparin or LMWH

A

Unfractionated heparin - contains pentasaccharide that binds to antithrombin causing confirmational change that increases ability to inactivate Factor Xa
- is able to bind to both antithrombrin and thrombrin to allow antithrombrin to inactivate thrombrin

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202
Q

Effect modification (of study)

A
  • is present when effect of main exposure on outcome is modified by presence of another variable. Effect modificatiion is not a bias.
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203
Q

Atherosclerosis

A

initiated by repetitive ENDOTHELIAL cell injury, which leads to chronic inflammatory state in underlying intima of large elastic arteries as well as large and medium-size elastic arteries

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204
Q

CML vs. leukemoid reaction

A
  • both have increased WBC count
  • differentiated based on neutrophil alkaline phosphatase level
  • in leukemoid reaction (decreased alkaline phosphotase)
  • in CML: decreased alkaline phosphatase
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205
Q

Causes of isolated systolic hypertension

A
  • caused by age-related decrease in compliance (increased stiffness) of the aorta and its proximal major branches
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207
Q

Discuss protein C deficiency and Warfarin therapy

A

Transient pro-coagulant/anti-coagulant imbalance is present at start of warfarin therapy , leading to relative hypercoaguable state with thrombotic occlusion of microvasculature and skin necrosis

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207
Q

Sx: urinary urgency, nocturia, and hesistancy associated with constant back pain in elderly main

A
  • Suggestive of metastatic prostate cancer

- Prostate cancer likes to metastasize to bone

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209
Q

Prostate cancer

A
  • adenocarcinomas in PERIPHERAL zone of gland (vs. periurethral zone of gland in BPH)
  • increased levels of PSA
  • in advanced disease, may see elevated prostatic acid phosphatase (tumor penetrates capsule) and increase alkaline phosphatase
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210
Q

Discuss obstructive sleep apnea and EPO secretion

A

Obstructive sleep apnea - recurrent episodes of aphyxia during sleep
- peritubular cells in renal cortex sense hypoxia and release EPO which increases hematocrit

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211
Q

Ovarian cancer

A
  • presents as ovarian mass
  • possible amenorrhea
  • associated with CA-125
  • risks of cancer decreased by OCP, multiparity, and breast feedings
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212
Q

Factors which decrease ovarian and/or endometrial cancers

A

** Things that decrease the number of ovulatory cycles
Multiparity
OCP use
Breastfeeding

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213
Q

Sx of hemorrhagic cystitis

A
  • can be result of nitrogen mustard based chemotherapeutic agent (e.g. cyclophosophamide or ifosfamide)
  • metabolized by kidneys into ACROLEIN, which toxic to uroepithelial cells and cause renal cell death and necrosis
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214
Q

Tx for hemorrhagic cystitis due to nitrogen mustard based chemotherapeutic agents

A

Mesna, a sulfhydryl compound that binds acrolein in urine

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215
Q

Osler-Weber-Rendu syndrome

A

autosomal dominant condition marked by telangiectasias in skin as well as in mucous membranes of lips, oranonasopharynx, respiratory tract, GI, and urintary tract
- may cause epitaxis, GI bleeding or hematuria

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216
Q

Tx of DVT in pregnant women

A

Heparin
- increases antithrombrin III activity

** Cant use Warfarin because it’s teratogenic

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217
Q

Clopidogrel

A

inhibits ADP mediated platelet aggeregation

used in treatment of coronary artery disease, peripheral vascular disease and cerebrovascular disease NOT DVT

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218
Q

Tx for diabetic ketoacidosis

A

Intravenous hydration with normal saline and insulin

- This will result in serum glucose, osmolality, and potassium as increases in serum HCO3 and sodium

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219
Q

Phentolamine

A
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220
Q

Phenylephrine

A
  • selective alpha adrenerigc agonist. Administation may be associated with reflect bradycardia
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221
Q

Isoproterenol

A

non-selective Beta adrenergic agonist that stimulates both B2 receptors vascular smooth muscle (vasodilation) and cardiac B1 (increases in cardiac rate and contractility)

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222
Q

Epinepherine (effect on systolic blood pressure, HR, and diastolic blood pressure)

A 
Systolic BP: increases (alpha 1 and B 1)
HR: increases (B1)
Diastolic BP: (dose-dependent)
Low dose - B2 > a1 (decrease)
High dose - a1 > B2 (increase)
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223
Q

Cachexia

A

syndrome that encompasses anorexia, malaise, anemia, weight loss and generalized wasting due to underlying systemic disease.

TNF-alpha is a cytokine that causes necrosis of some tumors in utero
- Inhibitig

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224
Q

IVC

A

formed by union of right and left common illiac veins at level of L4-L5

  • Renal arteries lie at L1
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225
Q

Tx for hyperlipidemia

A

Niacin (Vitamin B3) - decreases synthesis of hepatic triglyceride and VLDL by suppressing release of FA from peripheral tissues

  • also decreases VLDL conversion to LDL, thus decreasing LDL
  • also increases HDL
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226
Q

Discuss protein C deficiency and Warfarin therapy

A

Transient pro-coagulant/anti-coagulant imbalance is present at start of warfarin therapy , leading to relative hypercoaguable state with thrombotic occlusion of microvasculature and skin necrosis

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227
Q

Factors which decrease ovarian and/or endometrial cancers

A

** Things that decrease the number of ovulatory cycles
Multiparity
OCP use
Breastfeeding

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228
Q

Propranolol

A

non-selective beta adrenergic antagonist
effect on B2: vasoconstriction
effect on B1: slows heart contractility

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229
Q

Cachexia

A

syndrome that encompasses anorexia, malaise, anemia, weight loss and generalized wasting due to underlying systemic disease.

TNF-alpha is a cytokine that causes necrosis of some tumors in utero
- Inhibitig

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230
Q

IVC

A

formed by union of right and left common illiac veins at level of L4-L5

  • Renal arteries lie at L1
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231
Q

Explain relationship between blood flow and resistance

A

Blood flow is directly proportional to vessel radius raised to fourth power.

Resistance to blood flow is inversely proportional to vessel radius raised to fourth power.

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232
Q

Cystic medial degeneration

A
  • myxomatous changes in media of large arteries which development of aortic dissections and aortic aneurysms
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233
Q

Cachexia

A

syndrome that encompasses anorexia, malaise, anemia, weight loss and generalized wasting due to underlying systemic disease.

TNF-alpha is a cytokine that causes necrosis of some tumors in utero
- Inhibitig

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234
Q

IVC

A

formed by union of right and left common illiac veins at level of L4-L5

  • Renal arteries lie at L1
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235
Q

Li-Fraumeni syndromee

A
  • autosomal dominant disorder caused by inherited mutation of p53
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236
Q

Cyclin D1 overexpression

A
  • associated with Mantle lymphoma

- associated with t(11;14)

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237
Q

Migratory thrombophlebitis

A
  • should always think cancer
  • hypercoagulability is very common paraneoplastic syndrome seen in adenocarcinomas of pancrease, colon or lug
  • adenocarcinomas produce thromboplastin-like substance capable causing chronic intravascular coagulations
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238
Q

Fatty streaks

A
  • multiple yellow spots approx 1mm in diam
  • earliest lesion of atherosclerosis and present in all people > 10 y.o
  • composed of lipid filled foamy cells and smooth muscle cells that have engulfed lipoproteins
  • occurrence and location in child don’t predict locations of plaques later on in lide
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239
Q

Factor Xa inhibitors

A
  • new class of anticoagulants with activity against factor Xa
  • increase prothrombrin and aPTT times but don’t affect thrombrin time
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240
Q

Schilling test

A
  • helps differentiate between dietary defiency of vitamin B12, pernicious anemia, and malabsorption syndromes

** Low absorpton of cobalamin not correctable by IF means malabsorptive syndrome such as ileal disease, pancreatic insufficiency, or bacterial overgrowth

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241
Q

Sx of steroid abuse

A

erythrocytosis
abnormal testicle size and function
hepatic abnormalities
acne

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242
Q

Strong evidence of lymph node malignancy

A

Monoclonal lymphocytic proliferation, esp. monoclonal T-cell receptor gene arrangements

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243
Q

Polyclonal proliferation of lymphocytes

A

associated with benign lymph node enlargement in response to antigenic stimulation

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244
Q

Sx of lead poisoning

A
  • erythrocyte basophillic stippling

- microcytic anemia

245
Q

Net filtration pressure

A

(Gh - Bh) - (Go - Bo)

246
Q

von Willebrand disease

A
  • causes impairments in platelet function and coagulation pathway abnormalities.
  • associated with prolonged bleeding time
  • prolonged PTT
  • decreased platelet aggregation in response to ristocetin
247
Q

Polycythemia vera

A

clonal myeloproliferative disease in pluripotent stem cells

  • increased RBC mass, inc. plasma volume and low EPO
  • majority have JAK2 V617F mutation, rendering RBCs more sensitive to growth factors
248
Q

Strawberry hemangioma

A

benign vascular tumors of childhood

  • appear during first few weeks of life, initially grow rapidly, then regress by 5-8 years old
  • composed of capillaries separated by connective tissue
249
Q

Alpha adrenergic agonists

A
  • increase both systolic and diastolic blood pressure via stimulation of a-1 adrenoreceptors
  • elevated systemic blood pressure is associated with reflex increase in vagal influence on heart, results in decreased heart rate
250
Q

Vascular reaction to endothelial and intimal injury (e.g. atherosclerosis)

A

Intimal hyperplasia and fibrosis, predominantly mediated by reactive smooth muscle cells that migrate from media to the intima

251
Q

Nitroprusside

A
  • agent of choice in treating hypertensive emergency
  • quick onset of action and short duration of action
  • initially metabolized to cyanide , with conversion to thocyonate by liver rhodanase
252
Q

Treatment for nitroprusside toxicity

A

Na thiosulfate used to treat cyanide toxicity and works by donating sulfur to liver rhodamase to enhance conversion of cyanide to thiosulfate

253
Q

Likely diagnosis:
necrotizing vasculitis of upper and lower respiratory tract (nasal ulcerations, sinusitis, hemoptysis) and rapidly progressive Wegners

A
  • Wegner’s granulomatosis (granulomatous with polyangitis)

- cANCA

254
Q

Tx for familial hyoalphalipoproteinemia

A
  • low HDL thus increased risk of CAD

- Niacin (B3) is best treatment

255
Q

Tx for hypercholesteremia

A

Statins and ezetimbe

- High LDL is a risk factor for atherogenesis

256
Q

Ezetimbe

A
  • tx of hypercholestermia
  • decreases LDL by inhibiting cholesterol absorption from GI tract
  • does not increase HDL concentrations
  • used in combo with statins to achieve greater LDL reduction
257
Q

Statins

A
  • treatment of hypercholesteremias by inhibiting HMG CoA reductase, rate limiting enzyme of cholesterol syntehsis
  • in response hepatocytes, INCREASE expression of LDL receptors
258
Q

ApoB

A

apoprotein present in VLDL and LDL

- decreases in VLDL and LDL will cause apolipoprotein to decrease

259
Q

Discuss energy expenditure in fatty acid oxidation vs. glycolysis in cardiac function

A
  • More energy needed for fatty acid oxidation than glycolysis
  • inhibition of fatty acid inhibitors promote oxygen effiency and decreases oxygen needed for cardiac support
260
Q

Energy for sources of cardiac function

A
  • Glycolysis
  • Glucose oxidation
  • Fatty acid synthesis
261
Q

Main source of energy production in cardiac function

A

Fatty acid oxidation > Glucose oxidation > Glycolysis

262
Q

Varicose veins

A
  • caused by incompetent calves in lower extremities allow retrograde blood flow from deep to superficial veins
  • increases pressure in superficial veins –> dilated veins
263
Q

Orthostatic hypotension

A
  • defined as fall > 20 mmHg in systolic pressure or >10 mm Hg in diastolic pressure
264
Q

Describe events to correct normal physiological “orthostatic hypotension”

A
  1. Decreased venous return to heart
  2. Decreased ventricular filling and drop in CO and BP
  3. Drop in BP evokes compensatory baroreceptor reflect (increased sympathetic activity and decreased PS activity)
  4. Increased sympathetic tone raises peripheral vascular resistance via ALPHA-1 of peripheral vasculature. Leads to venous return and CO
265
Q

Understimulaion of alpha adrenoreceptorss

A
  • leads to cerebral hypoperfusion (light headedness and syncope
266
Q

Sinus venosus

A

forms smooth portion of right atrium

267
Q

Bulbus cordis

A

forms smooth portions of right and left ventricles

268
Q

Primitive atrium

A

forms rough portions of left and right atrium

269
Q

ductus arterious

A

derived from 6th embryonic aortic arch

270
Q

Tertiary syphillis

A
  • can result in thoracic aortic aneurysm
  • compresses adjacent structures and dilates aortic valve ring, a murmur and mediastinal widening is heard
  • aneurysm begins with vaso vasorum endarteritis and obliteration resulting in inflamation
271
Q

FTA-ABS

A

specific test for syphillis

272
Q

Gemfibrozil and fenofibrate (Fibric acid derivatives)

A
  • first line treatments for hyperTRIglyceridemia

- decrease serum triglycerides by reducing hepatic triglyceride production

273
Q

Bile acid resins (e.g. cholestyramine, colestpiol, colesevelam) and effect on triglycerides

A
  • increase triglycerides
274
Q

Niacin (Vitamin B3) used to treat…

A

hyperlipidemia

Side effects: cutaneous (flushing, warmth, itching) - better tolerated in slow release preparations

275
Q

Capsaicin

A
  • reduces pain by decreasing substance P in the peripeheral nervous system
276
Q

Buerger’s disease

A
  • seen in heavy cigarrete smokers < 35 years old
  • associated with intradermal injection of tobacco extracts
  • thrombosing vascultis extends into contiguous veins and nerves, encasing them in fibrous tissue
277
Q

Polyarteritis Nodosa

A
  • segmental, transmural, necrotizing inflammation of medium to small sized arteries in any organ
  • typically affects kidney, liver, heart, and GI tract but NOT LUNGS!!
  • sx: livedo reticularis, palpable purpura, hemorhage, ischemia
278
Q

Henoch-Schonlein purpura

A
  • seen in young boys (3 - 10 years old)
  • palpable skin lesions associated with abdominal pain, arthralgias, and renal involvement
  • occurs due to deposition of Ig-A containing immune complexes and shows up in purpura on buttocks, legs
279
Q

Glioblastoma multiforme

A
  • pallisading tumor cells around areas of necrosis
  • commonly crosses midline (“butterfly distribution”)
  • most common primary brain tumor in adults
280
Q

AML (M3 variant)

A
  • associated with Auer rods
  • t(15;17) which codes for chimeric PML/RAR (retinoic acid receptor)
  • this inhibits differentiation of myeloblasts and triggers development of APML
281
Q

Selective COX-2 inhibitors

A

e. g. celecoxib
- have no effect on platelet aggregation and cause little GI irritation
- the reduced risks of GI bleeding are only advantages over traditional NSAIDS

282
Q

Sx: “Mismatched V/Q with perfusion defect, sudden onset chest pain

A

Signs of pulmonary embolism

- often caused by deep vein thrombosis and continuum of pulmonary thromboembolism

283
Q

C-myc overexpression

A
  • associated with Burkitt’s lymphoma
  • associated with t(8;14)
  • myc oncogene on chr 8
  • Ig heavy chain on chr 14
284
Q

Greater activity against Factor Xa and Thrombrin.

Unfractionated heparin or LMWH

A

Unfractionated heparin - contains pentasaccharide that binds to antithrombin causing confirmational change that increases ability to inactivate Factor Xa
- is able to bind to both antithrombrin and thrombrin to allow antithrombrin to inactivate thrombrin

285
Q

Cystic medial degeneration

A
  • myxomatous changes in media of large arteries which development of aortic dissections and aortic aneurysms
286
Q

Ovarian cancer

A
  • presents as ovarian mass
  • possible amenorrhea
  • associated with CA-125
  • risks of cancer decreased by OCP, multiparity, and breast feedings
287
Q

Factors which decrease ovarian and/or endometrial cancers

A

** Things that decrease the number of ovulatory cycles
Multiparity
OCP use
Breastfeeding

288
Q

Sx of hemorrhagic cystitis

A
  • can be result of nitrogen mustard based chemotherapeutic agent (e.g. cyclophosophamide or ifosfamide)
  • metabolized by kidneys into ACROLEIN, which toxic to uroepithelial cells and cause renal cell death and necrosis
289
Q

Osler-Weber-Rendu syndrome

A

autosomal dominant condition marked by telangiectasias in skin as well as in mucous membranes of lips, oranonasopharynx, respiratory tract, GI, and urintary tract
- may cause epitaxis, GI bleeding or hematuria

290
Q

Tx for diabetic ketoacidosis

A

Intravenous hydration with normal saline and insulin

- This will result in serum glucose, osmolality, and potassium as increases in serum HCO3 and sodium

291
Q

Phentolamine

A
292
Q

Phenylephrine

A
  • selective alpha adrenerigc agonist. Administation may be associated with reflect bradycardia
293
Q

Isoproterenol

A

non-selective Beta adrenergic agonist that stimulates both B2 receptors vascular smooth muscle (vasodilation) and cardiac B1 (increases in cardiac rate and contractility)

294
Q

Epinepherine (effect on systolic blood pressure, HR, and diastolic blood pressure)

A 
Systolic BP: increases (alpha 1 and B 1)
HR: increases (B1)
Diastolic BP: (dose-dependent)
Low dose - B2 > a1 (decrease)
High dose - a1 > B2 (increase)
295
Q

Propranolol

A

non-selective beta adrenergic antagonist
effect on B2: vasoconstriction
effect on B1: slows heart contractility

296
Q

Treatment for nitroprusside toxicity

A

Na thiosulfate used to treat cyanide toxicity and works by donating sulfur to liver rhodamase to enhance conversion of cyanide to thiosulfate

297
Q

Likely diagnosis:
necrotizing vasculitis of upper and lower respiratory tract (nasal ulcerations, sinusitis, hemoptysis) and rapidly progressive Wegners

A
  • Wegner’s granulomatosis (granulomatous with polyangitis)

- cANCA

298
Q

Tx for familial hyoalphalipoproteinemia

A
  • low HDL thus increased risk of CAD

- Niacin (B3) is best treatment

299
Q

Tx for hypercholesteremia

A

Statins and ezetimbe

- High LDL is a risk factor for atherogenesis

300
Q

Ezetimbe

A
  • tx of hypercholestermia
  • decreases LDL by inhibiting cholesterol absorption from GI tract
  • does not increase HDL concentrations
  • used in combo with statins to achieve greater LDL reduction
301
Q

Statins

A
  • treatment of hypercholesteremias by inhibiting HMG CoA reductase, rate limiting enzyme of cholesterol syntehsis
  • in response hepatocytes, INCREASE expression of LDL receptors
302
Q

ApoB

A

apoprotein present in VLDL and LDL

- decreases in VLDL and LDL will cause apolipoprotein to decrease

303
Q

Discuss energy expenditure in fatty acid oxidation vs. glycolysis in cardiac function

A
  • More energy needed for fatty acid oxidation than glycolysis
  • inhibition of fatty acid inhibitors promote oxygen effiency and decreases oxygen needed for cardiac support
304
Q

Energy for sources of cardiac function

A
  • Glycolysis
  • Glucose oxidation
  • Fatty acid synthesis
305
Q

Main source of energy production in cardiac function

A

Fatty acid oxidation > Glucose oxidation > Glycolysis

306
Q

Varicose veins

A
  • caused by incompetent calves in lower extremities allow retrograde blood flow from deep to superficial veins
  • increases pressure in superficial veins –> dilated veins
307
Q

Orthostatic hypotension

A
  • defined as fall > 20 mmHg in systolic pressure or >10 mm Hg in diastolic pressure
308
Q

Describe events to correct normal physiological “orthostatic hypotension”

A
  1. Decreased venous return to heart
  2. Decreased ventricular filling and drop in CO and BP
  3. Drop in BP evokes compensatory baroreceptor reflect (increased sympathetic activity and decreased PS activity)
  4. Increased sympathetic tone raises peripheral vascular resistance via ALPHA-1 of peripheral vasculature. Leads to venous return and CO
309
Q

Understimulaion of alpha adrenoreceptorss

A
  • leads to cerebral hypoperfusion (light headedness and syncope
310
Q

Sinus venosus

A

forms smooth portion of right atrium

311
Q

Bulbus cordis

A

forms smooth portions of right and left ventricles

312
Q

Primitive atrium

A

forms rough portions of left and right atrium

313
Q

ductus arterious

A

derived from 6th embryonic aortic arch

314
Q

Tertiary syphillis

A
  • can result in thoracic aortic aneurysm
  • compresses adjacent structures and dilates aortic valve ring, a murmur and mediastinal widening is heard
  • aneurysm begins with vaso vasorum endarteritis and obliteration resulting in inflamation
315
Q

FTA-ABS

A

specific test for syphillis

316
Q

Gemfibrozil and fenofibrate (Fibric acid derivatives)

A
  • first line treatments for hyperTRIglyceridemia

- decrease serum triglycerides by reducing hepatic triglyceride production

317
Q

Bile acid resins (e.g. cholestyramine, colestpiol, colesevelam) and effect on triglycerides

A
  • increase triglycerides
318
Q

Niacin (Vitamin B3) used to treat…

A

hyperlipidemia

Side effects: cutaneous (flushing, warmth, itching) - better tolerated in slow release preparations

319
Q

Capsaicin

A
  • reduces pain by decreasing substance P in the peripeheral nervous system
320
Q

Buerger’s disease

A
  • seen in heavy cigarrete smokers < 35 years old
  • associated with intradermal injection of tobacco extracts
  • thrombosing vascultis extends into contiguous veins and nerves, encasing them in fibrous tissue
321
Q

Polyarteritis Nodosa

A
  • segmental, transmural, necrotizing inflammation of medium to small sized arteries in any organ
  • typically affects kidney, liver, heart, and GI tract but NOT LUNGS!!
  • sx: livedo reticularis, palpable purpura, hemorhage, ischemia
322
Q

Cachexia

A

syndrome that encompasses anorexia, malaise, anemia, weight loss and generalized wasting due to underlying systemic disease.

TNF-alpha is a cytokine that causes necrosis of some tumors in utero
- Inhibitig

323
Q

IVC

A

formed by union of right and left common illiac veins at level of L4-L5

  • Renal arteries lie at L1
324
Q

Apoptosis

A

can occur either the intrinsic (mitochondria-mediated) pathway or extrinsic (receptor-initiated) pathway.
- Both pathways require activation of caspases that cleave cellular proteins

325
Q

Explain relationship between blood flow and resistance

A

Blood flow is directly proportional to vessel radius raised to fourth power.

Resistance to blood flow is inversely proportional to vessel radius raised to fourth power.

326
Q

Foamy urine

A 
- caused by proteinuria/
Heavy proteinuria (e.g. nephrotic syndrome) can caused interstitial edema because of decrease in serum albumn
327
Q

Auer rods

A

deformed azurophilic granules found in cytoplasm of myeloblasts that stain positively for MYELOPEROXIDASE

  • found in abundance in AML M3
328
Q

Paroxysmal Nocturnal Hemoglobinuria

A

Triad: hemolytic anemia, hypercoaguable state, pancytopenia

  • causes thrombosis
  • deficiecy of CD55 and 59 which disallows complement activation
329
Q

Hereditary spherocytosis

A

autosomal dominant condition that results form red blood cell cytoskeletaon abnormalities

  • most commonly spectrin and ankrin defects
  • definitive test: positive osmotic fragility test
330
Q

Tx for hyperlipidemia

A

Niacin (Vitamin B3) - decreases synthesis of hepatic triglyceride and VLDL by suppressing release of FA from peripheral tissues

  • also decreases VLDL conversion to LDL, thus decreasing LDL
  • also increases HDL
331
Q

Bile-acid binding resins

A

can cause constipation and abdominal bloating (potentiall worsening diverticulosis), hypertriglyceridemia, cholesteral gall stone and vitamin K malabsorption

332
Q

Factor V Leiden mutation

A

causes factor Va resistance to inactivation by activated protein C, may account for approximately 20% of cases in atypcial venous thrombosis

333
Q

Sx of hereditary spherocytosis

A

anemia, reticulocytosis and increased indirect bilirubin, indicating hemolytic anemia

  • MCHC (increased because of mild dehydration of red blood cell)
  • blood smear dysmorphic red cells with no central pallor
334
Q

Microangiopathic hemolytic anemia

A
  • associaed with RBC fragments, Burr cells, and helmet cells

- in patients with prosthetic valves, RCs are exposed to excessive shear and turbulence in circulation causing damage

335
Q

Glomangioma

A
  • very tender small red-blue lesion under nail bed

- originates from modified smooth muscle cells that control thermoregulatory functions of dermal glomus bodies

336
Q

Bile acid-binding resins (cholestyramine, colestpiol, colesevelam)

A
  • bind to bile acid in GI tract, interfereing with its enterohepatic circulation
  • LDL is decreased b/c hepatic cholesterol is consumed in re-sythesis of bile acids
  • since bile is being re-synthesized, cholesterol content of bile increases thus increased risk of gallstone
337
Q

Bcl-abl

A
  • associated with CML

- result of t(9;22) - Philadelphia chromosome

338
Q

Li-Fraumeni syndromee

A
  • autosomal dominant disorder caused by inherited mutation of p53
339
Q

Bcl-2 overexpression

A
  • associated with Follicular lymphoma
  • associated with t(14;18)
  • Bcl-2 has anti-apoptotic effects
340
Q

Cyclin D1 overexpression

A
  • associated with Mantle lymphoma

- associated with t(11;14)

341
Q

Effect modification (of study)

A
  • is present when effect of main exposure on outcome is modified by presence of another variable. Effect modificatiion is not a bias.
342
Q

Atherosclerosis

A

initiated by repetitive ENDOTHELIAL cell injury, which leads to chronic inflammatory state in underlying intima of large elastic arteries as well as large and medium-size elastic arteries

343
Q

CML vs. leukemoid reaction

A
  • both have increased WBC count
  • differentiated based on neutrophil alkaline phosphatase level
  • in leukemoid reaction (decreased alkaline phosphotase)
  • in CML: decreased alkaline phosphatase
344
Q

Causes of isolated systolic hypertension

A
  • caused by age-related decrease in compliance (increased stiffness) of the aorta and its proximal major branches
345
Q

Discuss protein C deficiency and Warfarin therapy

A

Transient pro-coagulant/anti-coagulant imbalance is present at start of warfarin therapy , leading to relative hypercoaguable state with thrombotic occlusion of microvasculature and skin necrosis

346
Q

Sx: urinary urgency, nocturia, and hesistancy associated with constant back pain in elderly main

A
  • Suggestive of metastatic prostate cancer

- Prostate cancer likes to metastasize to bone

347
Q

Prostate cancer

A
  • adenocarcinomas in PERIPHERAL zone of gland (vs. periurethral zone of gland in BPH)
  • increased levels of PSA
  • in advanced disease, may see elevated prostatic acid phosphatase (tumor penetrates capsule) and increase alkaline phosphatase
348
Q

Discuss obstructive sleep apnea and EPO secretion

A

Obstructive sleep apnea - recurrent episodes of aphyxia during sleep
- peritubular cells in renal cortex sense hypoxia and release EPO which increases hematocrit

349
Q

Cystic medial degeneration

A
  • myxomatous changes in media of large arteries which development of aortic dissections and aortic aneurysms
350
Q

Ovarian cancer

A
  • presents as ovarian mass
  • possible amenorrhea
  • associated with CA-125
  • risks of cancer decreased by OCP, multiparity, and breast feedings
351
Q

Factors which decrease ovarian and/or endometrial cancers

A

** Things that decrease the number of ovulatory cycles
Multiparity
OCP use
Breastfeeding

352
Q

Tx for hemorrhagic cystitis due to nitrogen mustard based chemotherapeutic agents

A

Mesna, a sulfhydryl compound that binds acrolein in urine

353
Q

Tx of DVT in pregnant women

A

Heparin
- increases antithrombrin III activity

** Cant use Warfarin because it’s teratogenic

354
Q

Clopidogrel

A

inhibits ADP mediated platelet aggeregation

used in treatment of coronary artery disease, peripheral vascular disease and cerebrovascular disease NOT DVT

355
Q

Migratory thrombophlebitis

A
  • should always think cancer
  • hypercoagulability is very common paraneoplastic syndrome seen in adenocarcinomas of pancrease, colon or lug
  • adenocarcinomas produce thromboplastin-like substance capable causing chronic intravascular coagulations
356
Q

Nitroprusside

A
  • agent of choice in treating hypertensive emergency
  • quick onset of action and short duration of action
  • initially metabolized to cyanide , with conversion to thocyonate by liver rhodanase
357
Q

Treatment for nitroprusside toxicity

A

Na thiosulfate used to treat cyanide toxicity and works by donating sulfur to liver rhodamase to enhance conversion of cyanide to thiosulfate

358
Q

Likely diagnosis:
necrotizing vasculitis of upper and lower respiratory tract (nasal ulcerations, sinusitis, hemoptysis) and rapidly progressive Wegners

A
  • Wegner’s granulomatosis (granulomatous with polyangitis)

- cANCA

359
Q

Tx for familial hyoalphalipoproteinemia

A
  • low HDL thus increased risk of CAD

- Niacin (B3) is best treatment

360
Q

Tx for hypercholesteremia

A

Statins and ezetimbe

- High LDL is a risk factor for atherogenesis

361
Q

Ezetimbe

A
  • tx of hypercholestermia
  • decreases LDL by inhibiting cholesterol absorption from GI tract
  • does not increase HDL concentrations
  • used in combo with statins to achieve greater LDL reduction
362
Q

Statins

A
  • treatment of hypercholesteremias by inhibiting HMG CoA reductase, rate limiting enzyme of cholesterol syntehsis
  • in response hepatocytes, INCREASE expression of LDL receptors
363
Q

ApoB

A

apoprotein present in VLDL and LDL

- decreases in VLDL and LDL will cause apolipoprotein to decrease

364
Q

Discuss energy expenditure in fatty acid oxidation vs. glycolysis in cardiac function

A
  • More energy needed for fatty acid oxidation than glycolysis
  • inhibition of fatty acid inhibitors promote oxygen effiency and decreases oxygen needed for cardiac support
365
Q

Energy for sources of cardiac function

A
  • Glycolysis
  • Glucose oxidation
  • Fatty acid synthesis
366
Q

Main source of energy production in cardiac function

A

Fatty acid oxidation > Glucose oxidation > Glycolysis

367
Q

Varicose veins

A
  • caused by incompetent calves in lower extremities allow retrograde blood flow from deep to superficial veins
  • increases pressure in superficial veins –> dilated veins
368
Q

Orthostatic hypotension

A
  • defined as fall > 20 mmHg in systolic pressure or >10 mm Hg in diastolic pressure
369
Q

Describe events to correct normal physiological “orthostatic hypotension”

A
  1. Decreased venous return to heart
  2. Decreased ventricular filling and drop in CO and BP
  3. Drop in BP evokes compensatory baroreceptor reflect (increased sympathetic activity and decreased PS activity)
  4. Increased sympathetic tone raises peripheral vascular resistance via ALPHA-1 of peripheral vasculature. Leads to venous return and CO
370
Q

Understimulaion of alpha adrenoreceptorss

A
  • leads to cerebral hypoperfusion (light headedness and syncope
371
Q

Sinus venosus

A

forms smooth portion of right atrium

372
Q

Bulbus cordis

A

forms smooth portions of right and left ventricles

373
Q

Primitive atrium

A

forms rough portions of left and right atrium

374
Q

ductus arterious

A

derived from 6th embryonic aortic arch

375
Q

Tertiary syphillis

A
  • can result in thoracic aortic aneurysm
  • compresses adjacent structures and dilates aortic valve ring, a murmur and mediastinal widening is heard
  • aneurysm begins with vaso vasorum endarteritis and obliteration resulting in inflamation
376
Q

FTA-ABS

A

specific test for syphillis

377
Q

Gemfibrozil and fenofibrate (Fibric acid derivatives)

A
  • first line treatments for hyperTRIglyceridemia

- decrease serum triglycerides by reducing hepatic triglyceride production

378
Q

Bile acid resins (e.g. cholestyramine, colestpiol, colesevelam) and effect on triglycerides

A
  • increase triglycerides
379
Q

Niacin (Vitamin B3) used to treat…

A

hyperlipidemia

Side effects: cutaneous (flushing, warmth, itching) - better tolerated in slow release preparations

380
Q

Capsaicin

A
  • reduces pain by decreasing substance P in the peripeheral nervous system
381
Q

Buerger’s disease

A
  • seen in heavy cigarrete smokers < 35 years old
  • associated with intradermal injection of tobacco extracts
  • thrombosing vascultis extends into contiguous veins and nerves, encasing them in fibrous tissue
382
Q

Polyarteritis Nodosa

A
  • segmental, transmural, necrotizing inflammation of medium to small sized arteries in any organ
  • typically affects kidney, liver, heart, and GI tract but NOT LUNGS!!
  • sx: livedo reticularis, palpable purpura, hemorhage, ischemia
383
Q

Cachexia

A

syndrome that encompasses anorexia, malaise, anemia, weight loss and generalized wasting due to underlying systemic disease.

TNF-alpha is a cytokine that causes necrosis of some tumors in utero
- Inhibitig

384
Q

IVC

A

formed by union of right and left common illiac veins at level of L4-L5

  • Renal arteries lie at L1
385
Q

Apoptosis

A

can occur either the intrinsic (mitochondria-mediated) pathway or extrinsic (receptor-initiated) pathway.
- Both pathways require activation of caspases that cleave cellular proteins

386
Q

Explain relationship between blood flow and resistance

A

Blood flow is directly proportional to vessel radius raised to fourth power.

Resistance to blood flow is inversely proportional to vessel radius raised to fourth power.

387
Q

Foamy urine

A 
- caused by proteinuria/
Heavy proteinuria (e.g. nephrotic syndrome) can caused interstitial edema because of decrease in serum albumn
388
Q

Auer rods

A

deformed azurophilic granules found in cytoplasm of myeloblasts that stain positively for MYELOPEROXIDASE

  • found in abundance in AML M3
389
Q

Paroxysmal Nocturnal Hemoglobinuria

A

Triad: hemolytic anemia, hypercoaguable state, pancytopenia

  • causes thrombosis
  • deficiecy of CD55 and 59 which disallows complement activation
390
Q

Hereditary spherocytosis

A

autosomal dominant condition that results form red blood cell cytoskeletaon abnormalities

  • most commonly spectrin and ankrin defects
  • definitive test: positive osmotic fragility test
391
Q

Tx for hyperlipidemia

A

Niacin (Vitamin B3) - decreases synthesis of hepatic triglyceride and VLDL by suppressing release of FA from peripheral tissues

  • also decreases VLDL conversion to LDL, thus decreasing LDL
  • also increases HDL
392
Q

Bile-acid binding resins

A

can cause constipation and abdominal bloating (potentiall worsening diverticulosis), hypertriglyceridemia, cholesteral gall stone and vitamin K malabsorption

393
Q

Factor V Leiden mutation

A

causes factor Va resistance to inactivation by activated protein C, may account for approximately 20% of cases in atypcial venous thrombosis

394
Q

Sx of hereditary spherocytosis

A

anemia, reticulocytosis and increased indirect bilirubin, indicating hemolytic anemia

  • MCHC (increased because of mild dehydration of red blood cell)
  • blood smear dysmorphic red cells with no central pallor
395
Q

Henoch-Schonlein purpura

A
  • seen in young boys (3 - 10 years old)
  • palpable skin lesions associated with abdominal pain, arthralgias, and renal involvement
  • occurs due to deposition of Ig-A containing immune complexes and shows up in purpura on buttocks, legs
396
Q

Glioblastoma multiforme

A
  • pallisading tumor cells around areas of necrosis
  • commonly crosses midline (“butterfly distribution”)
  • most common primary brain tumor in adults
397
Q

AML (M3 variant)

A
  • associated with Auer rods
  • t(15;17) which codes for chimeric PML/RAR (retinoic acid receptor)
  • this inhibits differentiation of myeloblasts and triggers development of APML
398
Q

Selective COX-2 inhibitors

A

e. g. celecoxib
- have no effect on platelet aggregation and cause little GI irritation
- the reduced risks of GI bleeding are only advantages over traditional NSAIDS

399
Q

Microangiopathic hemolytic anemia

A
  • associaed with RBC fragments, Burr cells, and helmet cells

- in patients with prosthetic valves, RCs are exposed to excessive shear and turbulence in circulation causing damage

400
Q

Glomangioma

A
  • very tender small red-blue lesion under nail bed

- originates from modified smooth muscle cells that control thermoregulatory functions of dermal glomus bodies

401
Q

Sx: “Mismatched V/Q with perfusion defect, sudden onset chest pain

A

Signs of pulmonary embolism

- often caused by deep vein thrombosis and continuum of pulmonary thromboembolism

402
Q

Bile acid-binding resins (cholestyramine, colestpiol, colesevelam)

A
  • bind to bile acid in GI tract, interfereing with its enterohepatic circulation
  • LDL is decreased b/c hepatic cholesterol is consumed in re-sythesis of bile acids
  • since bile is being re-synthesized, cholesterol content of bile increases thus increased risk of gallstone
403
Q

Bcl-abl

A
  • associated with CML

- result of t(9;22) - Philadelphia chromosome

404
Q

C-myc overexpression

A
  • associated with Burkitt’s lymphoma
  • associated with t(8;14)
  • myc oncogene on chr 8
  • Ig heavy chain on chr 14
405
Q

Li-Fraumeni syndromee

A
  • autosomal dominant disorder caused by inherited mutation of p53
406
Q

Bcl-2 overexpression

A
  • associated with Follicular lymphoma
  • associated with t(14;18)
  • Bcl-2 has anti-apoptotic effects
407
Q

Cyclin D1 overexpression

A
  • associated with Mantle lymphoma

- associated with t(11;14)

408
Q

Greater activity against Factor Xa and Thrombrin.

Unfractionated heparin or LMWH

A

Unfractionated heparin - contains pentasaccharide that binds to antithrombin causing confirmational change that increases ability to inactivate Factor Xa
- is able to bind to both antithrombrin and thrombrin to allow antithrombrin to inactivate thrombrin

409
Q

Effect modification (of study)

A
  • is present when effect of main exposure on outcome is modified by presence of another variable. Effect modificatiion is not a bias.
410
Q

Atherosclerosis

A

initiated by repetitive ENDOTHELIAL cell injury, which leads to chronic inflammatory state in underlying intima of large elastic arteries as well as large and medium-size elastic arteries

411
Q

CML vs. leukemoid reaction

A
  • both have increased WBC count
  • differentiated based on neutrophil alkaline phosphatase level
  • in leukemoid reaction (decreased alkaline phosphotase)
  • in CML: decreased alkaline phosphatase
412
Q

Causes of isolated systolic hypertension

A
  • caused by age-related decrease in compliance (increased stiffness) of the aorta and its proximal major branches
413
Q

Discuss protein C deficiency and Warfarin therapy

A

Transient pro-coagulant/anti-coagulant imbalance is present at start of warfarin therapy , leading to relative hypercoaguable state with thrombotic occlusion of microvasculature and skin necrosis

414
Q

Sx: urinary urgency, nocturia, and hesistancy associated with constant back pain in elderly main

A
  • Suggestive of metastatic prostate cancer

- Prostate cancer likes to metastasize to bone

415
Q

Prostate cancer

A
  • adenocarcinomas in PERIPHERAL zone of gland (vs. periurethral zone of gland in BPH)
  • increased levels of PSA
  • in advanced disease, may see elevated prostatic acid phosphatase (tumor penetrates capsule) and increase alkaline phosphatase
416
Q

Discuss obstructive sleep apnea and EPO secretion

A

Obstructive sleep apnea - recurrent episodes of aphyxia during sleep
- peritubular cells in renal cortex sense hypoxia and release EPO which increases hematocrit

417
Q

Cystic medial degeneration

A
  • myxomatous changes in media of large arteries which development of aortic dissections and aortic aneurysms
418
Q

Ovarian cancer

A
  • presents as ovarian mass
  • possible amenorrhea
  • associated with CA-125
  • risks of cancer decreased by OCP, multiparity, and breast feedings
419
Q

Factors which decrease ovarian and/or endometrial cancers

A

** Things that decrease the number of ovulatory cycles
Multiparity
OCP use
Breastfeeding

420
Q

Sx of hemorrhagic cystitis

A
  • can be result of nitrogen mustard based chemotherapeutic agent (e.g. cyclophosophamide or ifosfamide)
  • metabolized by kidneys into ACROLEIN, which toxic to uroepithelial cells and cause renal cell death and necrosis
421
Q

Tx for hemorrhagic cystitis due to nitrogen mustard based chemotherapeutic agents

A

Mesna, a sulfhydryl compound that binds acrolein in urine

422
Q

Osler-Weber-Rendu syndrome

A

autosomal dominant condition marked by telangiectasias in skin as well as in mucous membranes of lips, oranonasopharynx, respiratory tract, GI, and urintary tract
- may cause epitaxis, GI bleeding or hematuria

423
Q

Tx of DVT in pregnant women

A

Heparin
- increases antithrombrin III activity

** Cant use Warfarin because it’s teratogenic

424
Q

Clopidogrel

A

inhibits ADP mediated platelet aggeregation

used in treatment of coronary artery disease, peripheral vascular disease and cerebrovascular disease NOT DVT

425
Q

Tx for diabetic ketoacidosis

A

Intravenous hydration with normal saline and insulin

- This will result in serum glucose, osmolality, and potassium as increases in serum HCO3 and sodium

426
Q

Phentolamine

A
427
Q

Phenylephrine

A
  • selective alpha adrenerigc agonist. Administation may be associated with reflect bradycardia
428
Q

Isoproterenol

A

non-selective Beta adrenergic agonist that stimulates both B2 receptors vascular smooth muscle (vasodilation) and cardiac B1 (increases in cardiac rate and contractility)

429
Q

Epinepherine (effect on systolic blood pressure, HR, and diastolic blood pressure)

A 
Systolic BP: increases (alpha 1 and B 1)
HR: increases (B1)
Diastolic BP: (dose-dependent)
Low dose - B2 > a1 (decrease)
High dose - a1 > B2 (increase)
430
Q

Propranolol

A

non-selective beta adrenergic antagonist
effect on B2: vasoconstriction
effect on B1: slows heart contractility

431
Q

Fatty streaks

A
  • multiple yellow spots approx 1mm in diam
  • earliest lesion of atherosclerosis and present in all people > 10 y.o
  • composed of lipid filled foamy cells and smooth muscle cells that have engulfed lipoproteins
  • occurrence and location in child don’t predict locations of plaques later on in lide
432
Q

Factor Xa inhibitors

A
  • new class of anticoagulants with activity against factor Xa
  • increase prothrombrin and aPTT times but don’t affect thrombrin time
433
Q

Schilling test

A
  • helps differentiate between dietary defiency of vitamin B12, pernicious anemia, and malabsorption syndromes

** Low absorpton of cobalamin not correctable by IF means malabsorptive syndrome such as ileal disease, pancreatic insufficiency, or bacterial overgrowth

434
Q

Sx of steroid abuse

A

erythrocytosis
abnormal testicle size and function
hepatic abnormalities
acne

435
Q

Strong evidence of lymph node malignancy

A

Monoclonal lymphocytic proliferation, esp. monoclonal T-cell receptor gene arrangements

436
Q

Polyclonal proliferation of lymphocytes

A

associated with benign lymph node enlargement in response to antigenic stimulation

437
Q

Sx of lead poisoning

A
  • erythrocyte basophillic stippling

- microcytic anemia

438
Q

Net filtration pressure

A

(Gh - Bh) - (Go - Bo)

439
Q

von Willebrand disease

A
  • causes impairments in platelet function and coagulation pathway abnormalities.
  • associated with prolonged bleeding time
  • prolonged PTT
  • decreased platelet aggregation in response to ristocetin
440
Q

Polycythemia vera

A

clonal myeloproliferative disease in pluripotent stem cells

  • increased RBC mass, inc. plasma volume and low EPO
  • majority have JAK2 V617F mutation, rendering RBCs more sensitive to growth factors
441
Q

Strawberry hemangioma

A

benign vascular tumors of childhood

  • appear during first few weeks of life, initially grow rapidly, then regress by 5-8 years old
  • composed of capillaries separated by connective tissue
442
Q

Alpha adrenergic agonists

A
  • increase both systolic and diastolic blood pressure via stimulation of a-1 adrenoreceptors
  • elevated systemic blood pressure is associated with reflex increase in vagal influence on heart, results in decreased heart rate
443
Q

Vascular reaction to endothelial and intimal injury (e.g. atherosclerosis)

A

Intimal hyperplasia and fibrosis, predominantly mediated by reactive smooth muscle cells that migrate from media to the intima

444
Q

Nitroprusside

A
  • agent of choice in treating hypertensive emergency
  • quick onset of action and short duration of action
  • initially metabolized to cyanide , with conversion to thocyonate by liver rhodanase
445
Q

Treatment for nitroprusside toxicity

A

Na thiosulfate used to treat cyanide toxicity and works by donating sulfur to liver rhodamase to enhance conversion of cyanide to thiosulfate

446
Q

Likely diagnosis:
necrotizing vasculitis of upper and lower respiratory tract (nasal ulcerations, sinusitis, hemoptysis) and rapidly progressive Wegners

A
  • Wegner’s granulomatosis (granulomatous with polyangitis)

- cANCA

447
Q

Tx for familial hyoalphalipoproteinemia

A
  • low HDL thus increased risk of CAD

- Niacin (B3) is best treatment

448
Q

Tx for hypercholesteremia

A

Statins and ezetimbe

- High LDL is a risk factor for atherogenesis

449
Q

Ezetimbe

A
  • tx of hypercholestermia
  • decreases LDL by inhibiting cholesterol absorption from GI tract
  • does not increase HDL concentrations
  • used in combo with statins to achieve greater LDL reduction
450
Q

Statins

A
  • treatment of hypercholesteremias by inhibiting HMG CoA reductase, rate limiting enzyme of cholesterol syntehsis
  • in response hepatocytes, INCREASE expression of LDL receptors
451
Q

ApoB

A

apoprotein present in VLDL and LDL

- decreases in VLDL and LDL will cause apolipoprotein to decrease

452
Q

Discuss energy expenditure in fatty acid oxidation vs. glycolysis in cardiac function

A
  • More energy needed for fatty acid oxidation than glycolysis
  • inhibition of fatty acid inhibitors promote oxygen effiency and decreases oxygen needed for cardiac support
453
Q

Energy for sources of cardiac function

A
  • Glycolysis
  • Glucose oxidation
  • Fatty acid synthesis
454
Q

Main source of energy production in cardiac function

A

Fatty acid oxidation > Glucose oxidation > Glycolysis

455
Q

Varicose veins

A
  • caused by incompetent calves in lower extremities allow retrograde blood flow from deep to superficial veins
  • increases pressure in superficial veins –> dilated veins
456
Q

Orthostatic hypotension

A
  • defined as fall > 20 mmHg in systolic pressure or >10 mm Hg in diastolic pressure
457
Q

Describe events to correct normal physiological “orthostatic hypotension”

A
  1. Decreased venous return to heart
  2. Decreased ventricular filling and drop in CO and BP
  3. Drop in BP evokes compensatory baroreceptor reflect (increased sympathetic activity and decreased PS activity)
  4. Increased sympathetic tone raises peripheral vascular resistance via ALPHA-1 of peripheral vasculature. Leads to venous return and CO
458
Q

Understimulaion of alpha adrenoreceptorss

A
  • leads to cerebral hypoperfusion (light headedness and syncope
459
Q

Sinus venosus

A

forms smooth portion of right atrium

460
Q

Bulbus cordis

A

forms smooth portions of right and left ventricles

461
Q

Primitive atrium

A

forms rough portions of left and right atrium

462
Q

ductus arterious

A

derived from 6th embryonic aortic arch

463
Q

Tertiary syphillis

A
  • can result in thoracic aortic aneurysm
  • compresses adjacent structures and dilates aortic valve ring, a murmur and mediastinal widening is heard
  • aneurysm begins with vaso vasorum endarteritis and obliteration resulting in inflamation
464
Q

FTA-ABS

A

specific test for syphillis

465
Q

Gemfibrozil and fenofibrate (Fibric acid derivatives)

A
  • first line treatments for hyperTRIglyceridemia

- decrease serum triglycerides by reducing hepatic triglyceride production

466
Q

Bile acid resins (e.g. cholestyramine, colestpiol, colesevelam) and effect on triglycerides

A
  • increase triglycerides
467
Q

Niacin (Vitamin B3) used to treat…

A

hyperlipidemia

Side effects: cutaneous (flushing, warmth, itching) - better tolerated in slow release preparations

468
Q

Capsaicin

A
  • reduces pain by decreasing substance P in the peripeheral nervous system
469
Q

Buerger’s disease

A
  • seen in heavy cigarrete smokers < 35 years old
  • associated with intradermal injection of tobacco extracts
  • thrombosing vascultis extends into contiguous veins and nerves, encasing them in fibrous tissue
470
Q

Polyarteritis Nodosa

A
  • segmental, transmural, necrotizing inflammation of medium to small sized arteries in any organ
  • typically affects kidney, liver, heart, and GI tract but NOT LUNGS!!
  • sx: livedo reticularis, palpable purpura, hemorhage, ischemia
471
Q

6-mercaptopurine

A
  • cytotoxic purine analog (along with 6-thioguanine) that inhibits de novo purine synthesis after being converted to active metabolites by hypoxanthine-gunine phosphoribosyl transferase (HGPRT)
  • inactivated by XANTHINE OXIDASE in liver
472
Q

Allopurinol

A
  • inhibits xanthine oxidase and thus can increase 6-mercaptopurine significantly
473
Q

Adenosine deaminase

A
  • converts adenosine to iosine as part of purine metabolims
  • enzyme is deficient in SCID
  • inhibited by Pentostatin
474
Q

Glioblastoma

A
  • tumor from astrocytes and is common adult tumor
  • typically found in hemispheres (frontal or temporal lobes) or near basal ganglia
  • may become large enough to cross midline
  • on gross exam: soft and poorly defined with areas of necrosis and hemorrhage
  • poor prognosis
475
Q

Meningiomas

A
  • derived from arachnoid cells and 2nd most common tumor
  • located on brain surface and attached to dura
  • found on lateral and parasagittal brain convexities, falx cerebri, sphenoidal ridge and olfactory groove
476
Q

Acoustic schwannomas

A
  • special type that are arise from Schwann cells of CN VIII

- found at cerebellopontine angle

477
Q

Precursor B-ALL immunophenotyping

A

can be distinguished from Precursor T-ALL by immunophenotyping

  • TdT+
  • CD10 +
  • CD19+
478
Q

Precursor T-ALL immunophenotyping

A 
- express T-cell markers and are TdT+
CD2
CD3
CD4
CD7
CD8
479
Q

Epstein-Barr Virus

A
  • responsible for acute mononucleosis, nasopharyngeal carcinoma, lymphomas, and Burkitt’s lymphoma
  • via contact through oropharyngeal secretions
  • binds to CD21 on surface of B-cells and epithelial cells
480
Q

Parvovirus

A
  • attaches to human epithelioid cells via blood group P antigen (globoside)
  • immature cells of erythroud family are most vulnerable and why fetal liver and adult bone marrow are most vulnerable
481
Q

Essential to viral invasion of cells

A

the initial attachment of virion envelope or capsid surface to complementary cell surface receptorss
(e.g CD4 and HIV gp120) OR CD21 and EBVgp350

482
Q

ERB-B2

A
  • overexpressed in 25 - 30% of breast cancers
  • member of epidermal growth factors
  • transmembraine protein with tyrosine kinase activity and is present in breast/ovarian cells
  • associated with aggressive cancers
483
Q

Stop codons

A

UAG - “U Are Gone
UGA - “U Go Away”
UAA - “U Are Away

484
Q

Treatment for cyanide poisoning

A

Amyl nitrates

  • cause methoglobinemia which contains Fe (2+)
  • Cyanide binds to Fe(2+)/ ferric iron more avidly than cytochrone enzymes which mitochrondrial enzymes
485
Q

Anaphylatic shock

A
  • characterized by vasodilation, increased vascular permeability, bronchoconstriction, hypotension, tachycardia
  • epinephrine is treatment for anaphylatic shock
486
Q

Aplastic anemia

A
  • causes pancytopenia as bone marrow is replaced with fat cells and fibrous stroma
  • absence of splenomegaly
  • need bone marrow biopsy to distinguish from myeloproliferative disease
487
Q

Polyarteritis Nodosa (PAN)

A
  • TRANSMURAL inflammation with arterial wall fibrinoid necrosis
  • sx;: fever, malaise, weight loss, abdominal pain and melensa
  • associated with Hep B infection
488
Q

Sx of norepinephrine extravasation

A
  • blanching of a vein into which norepinepherine is being infused together with induration and pallor of tissues surrounding IV site
  • due to leak of alpha-1 causing vasoconstriction
  • prevented by administering phentolamine mesylate
489
Q

Tx of norepinephrine extravasation

A
  • alpha 1 blocker (phentolamine)

- counteracts the alpha-1 receptor mediated vasocontriction

490
Q

Sx of aortic dissection

A
  • severe retrosternal pain that radiates to pack
  • intimal tear is thought to the initiating process
  • intramural hematoma dissects through media and can compress major arterial branches
491
Q

Vitamin K dependent coagulation factors

A
  • II, VII, IX, X - (which are initially produced in liver until activation by Vitamin K
  • failure of prolonged PT to correct with Vit K indicates liver disease
492
Q

DIC (Disseminate Intravascular Coagulation

A
  • associated with sepsis, particularly Gram - rods
  • occurs to exposure of blood to procoagulants that initiate intravascular thrombosis, which is followed by thrombolysis
  • result is consumption of coagulation factors, coagulopatjy, and bleeding
493
Q

TTP-HUS

A
    • TTP - associated with adults with neurological disorders
    • HUS - associated with children with predominant renal involvement
  • patients usually don’t bleed
  • ONLY platelets are activated
  • normal PT and PTT
  • normal fibrinogen
494
Q

DIC

A
  • patients bleed
  • coagulation cascade is activated
  • PT and PTT are prolonged
  • Low fibrinogen and increased FDP (fibrin degredation products)
495
Q

Sx: Hemophilia

A
  • history of hemarthrosis and bleeding after tooth extractions
  • due to decreased levels of factors VIII (Hemophilia A) or levels of factors IX (Hemophilia B) which lead to failure to convert prothrombrin to thrombrin and deficient thrombrin formation
  • Addition of thrombrin to hemophiliac patients’ serum causes clotting
496
Q

Cliostazol

A

phosphodiesterase inhibitor used in patients with intermittent claudication

  • inhibits platelet aggregation AND direct vasodilator
  • shown to be superior to asprin in treatment of peripheral arterial disease
497
Q

Ticlopidine / Clopidogrel

A
  • similar drugs that block ADP receptor thus preventing GPIIb/IIIa receptors from functioning (platelet aggregation)
498
Q

Argatroban

A
  • anti-thrombic drug largely used in treatment of heparin induced thrombocytopenia
499
Q

Concern when initiating ACE inhibitor therapy

A
  • first dose HYPOtension
  • risk factors: hyponatremia, hypovolemia secondary to diuretics, low baseline BP, high renin or aldosterone levels, renal impariemtn and heart failure
  • minimize risk with low doses
500
Q

Etoposide

A

chemotherapeutic agen that inhibits sealing activity of toperisomerase II.

  • treatment causes chromosomal break to accumulate in dividing cells, causing cell death
501
Q

Topoisomerases

A
  • enzymes that relieve supercoiling that occurs during DNA replicating by creating nicks
    Topoisomerase I – makes SINGLE stranded nicks
    Topoisomerase II - makes DOUBLE stranded nicks
502
Q

Most common VIRAL cause of aplastic crisis in patients with sickle cell anemia or chronic hemolytic anemia

A

Parvovirus B19 (non-enveloped, single stranded DNA virus) - infects erythroid progenitor cells

503
Q

Hepatic C

A
  • enveloped single stranded + RNA virus
504
Q

Hepatitis E

A

NON-ENVELOPED single stranded + RNA virus

505
Q

Paracortex of lymph node

A

populated by T lymphocytes and dendritic cells

  • internal to cortex between follicles and medulla)
  • can become enlarged during proliferation of T-lymphocytes
  • poorly developed in DiGeorge Syndrome
506
Q

Follicles of lymph node

A

sites of B cell lymphocyte localization and proliferation
- dense and dormant
- secondary follicles have pale germinal center containing proliferating B cells and follicular dendritic cells
-

507
Q

Discuss agammaglobulinemia and follicles of lymph nodes

A

In agammaglobulinemia, germinal centers and primary lymph node follicle don’t form due to absence of B cells

508
Q

Medulla of lymph node

A
  • consists of medullary cords and sinuses
  • medullary cords contain B cells, plasma cells, and macrophages
  • medullary sinuses contain reticular cells and macrophages
509
Q

Median

A

value that is located at the precise center of an ordered dataset. Divides the right half data from left side

510
Q

BRCA-1 and BRCA-2

A

inherited as an autosomal dominant trait
BRCA-1 on chromosome 17 and is a tumor suppressor gene
- should be suspected if two or more relatives have premenopausal breast cancer

511
Q

Hemolytic Uremic Syndrome

A
  • affects young children and manifests with acute renal failure microangiopathic hemolytic anemia and thrombocytopenia
  • decreased RBC count, hematocrine, platelet count
  • Increased bleedigng time, LDH, BUN, and creatiine
512
Q

Organisms associated with HUS

A
  • E.coli O157:H7

- Shigella

513
Q

Presence of erythroid precursors in liver and spleen..

A
  • Extramedullary hematopoiesis, condition characterized by erythropoietin-stimulated hyperplastic marrow cell invasion of extramedullar organs
  • caused by severed chronic hemolytic anemia, such as B-thalassemia
514
Q

APC gene mutation

A
  • found in patients with familial polyposis syndromes, sporadic colon cancer, and melenoma
  • responsible for maintaining low levels of B-catenin (oncogenic) ad for intercellular adhesion
515
Q

Non-nucleoside reverse transcriptase inhibitors

A

Nevirapine, Efavirenz, Delaviradine

516
Q

Non-nucleoside reverse transcriptase inhibitors (Nevirapine, Efavirenz, Delaviradine)

A
  • anti-retroviral drugs that do not require activation via intracellular phosphorylation
  • best used with other anti-retroviral agents in treatment of HIV
517
Q

Adverse effects of NNRTs (Nevirapine, Efiravenz, Delaviradine)

A
  • abrupt onset of flu-like symptoms, jaudine, or fever - may indicate hepatic failure with encephalopathy
  • most likely to occur during first 6 weeks of therapy
  • Stevens - Johnsons syndrome
518
Q

Pure red cell aplasia

A

Rare form of marrow failure characterized by severe hypoplasia of marrow erythroid elements in setting of normal granulopoiesis and thromboeisis

  • involves inhibition of erythropoietic precursors and progenitors by IgG auto-antibodies or T lymphocytes
519
Q

Pure red cell aplasia is associated with which conditions?

A
  • Thymoma
  • Lymphocytic leukemias
  • Parovirus B19 infection
520
Q

Volume of distribution

A
  • hypothetical volume of fluid into which administered amt of drug would be need to uniformly distributed to produced observed plasma concentration
521
Q

Volume of distribution formula

A
  • amount of drug (mg)/ plasma concentration of drug (mg/L)
522
Q

Low Vd (3-5L)

A
  • high molecular weight
  • high plasma protein binding
  • high charge
  • hydrophilicity tend to trap the drug
523
Q

High Vd

A
  • Lipophilic drugs tend to readily cross cell membranes and distribute widely outside the plasma and interstitial compartments
  • tendency to collect with cells gives these drugs a high volume of distrubution
524
Q

ARBs

A
  • by blocking AT-1 receptors and inhibiting effects of angiotensin II
  • results in arterial vasodilation and decreased aldosterone secretion
525
Q

Discuss AT-1 receptors and RAAS

A
  • Stimulation of AT-1 receptors on juxtaglomerular cells will decrease renin release from kidneys, consequently decreasing angiotensin I and II levels
526
Q

Attributable risk percent

A
  • measures of impact of a risk factor being studies
    = (risk in exposed - risk in unexposed)/ risk in exposed

ARP = (RR - 1)/RR

527
Q

Sickle cell patients are at increased risk for infection by which organisms

A
  • Encaspsulated organisms (Strep pneumoniae and H. influenzae)
  • Salmonells is most common cause of osteomyelitis, followed by E. coil and S. aureus
528
Q

Reed Sternberg cells

A

bi-nucleated cells with “owl’s eyes” appearance that appear on background of lymphocytic infiltrate
- seen in non-Hodgkin lymphoma

529
Q

Most common cancers in women

A

Incidence : Breast > Lung > Colon

Mortality: Lung > Breast > Colon

530
Q

Rituximab

A
  • monoclonal antibody directed against CD20 (B-cell marker) antigen.
  • introduction of prognosis of some lymphomas
531
Q

P450 microsomal oxidase system

A
  • plays an important role in detoxification

- CCl(4) - produces free radicals that start vicious cycle of hepatic injury

532
Q

Henoch Schonlein Purpura

A
  • small vessel leukocytoclastic angiitis associated with IgA and C3 deposition
  • most common in children in 3 - 11 years old
  • most children with palpable skin lesions with or without abdominal pain and athralgias
533
Q

Anti-retroviral agents

A
  • selectively bind to HIV envelope transmembrane glycoprotein gp41 prevent the conformational changes necessary for viral membrane to fuse with target cellular membrane
  • “fusion inhibitor”
534
Q

Heparin

A

drug of choice for acute management of venous thrombosis or thromboembolism
- activates antithrombin III

535
Q

Warfarin

A

inhibits Vitamin K dependent-carboxylation of glutamic acid residues of clotting factors II, VII, IX, and X (vitamin K dependent clotting factors)

536
Q

Discuss ACE inhibitors and patients with bilateral renal stenosis

A
  • May cause drop in GFR, as ACE inhibition leads to vasodilation of EFFERENT arterioles
537
Q

Hyperplastic arteriosclerosis

A
  • can result from perpetual malignant hypertension
  • diastolic blood pressure > 120 - 130 mmHg
  • leads to onion-like concentric thickening of walls of arterioles as result of laminated smooth muscle cells and reduplicated BM
  • seen in renal vasculature
538
Q

Hereditary spherocytosis

A
  • results from RBC cytoskeleton abnormalities (deficient in ankyrin, spectrin)

Sx: hemolytic anemia, jaundice and splenomegaly

539
Q

Aplastic anemia

A
  • characterized by panocytopenia, low reticulocyte count, and absent splenomegaly
  • usually “dry” and histopathology shows marrow replacement with fat cells and fibrous stroma
540
Q

Myelofibrosis

A
  • replacement of bone marrow with FIBROUS tissue (no fat)
  • pancytopenia
  • evidence of extramedullary hematopoiesis
541
Q

Hairy leukemia

A
  • can bone marrow fibrosis
  • often described as “fried egg” appearance
  • evidence of splenomegaly
542
Q

Chloramphenicol

A
  • lead to both dose-dependent (reversible) and dose-dependent (irreversible) pancytopenia
  • anti-bacterial by binding to ribosomal 50 subunit and inhibiting peptidyl transferase enzyme thus suppresses bacterial synthesis
543
Q

Gentamicin side efects

A
  • Ototoxicity
  • Nephrotoxicity
  • Neuromusclar paralysis (
544
Q

Metronidazole side effect

A
  • Side effects are GI (nausea, vomiting, crampy abdominal pain)
  • neurologica (parasthsias, dizziness)
  • induces disulfiram-like reaction
545
Q

Vancomycin-side effect

A

Can cause histamine release, results in flushing
“red man syndrome” - cause by rapid infusin

  • dose-related ototoxicity
546
Q

Common cardinal veins make what post-natal structure?

A

SVC

547
Q

EBV virus

A
  • causes infectious mononucleosis
  • sx: sore throat, cervical lymphadenopathy, splenomegaly
  • heterophile antibody tests sensitive
  • associated with Burkitt lymphoma (African type) and nasopharyngeal carcinoma (in Cinese)
548
Q

Features of anaplasia

A
  • loss of cell polarity and normal tissue architecture
  • variation in shape and size
  • hyperchromatism and nuclear pleomorphisn
  • High nucleus to cytoplasm ration
  • Numerous, often abnormal mitoses
  • giant, multi-nucleated tumor cells
549
Q

Southwestern blotting

A

technique that analyzes DNA- binding proteins using pricincples of Southern and Western bots
- DNA- binding proteins recognized by ability to ind to specific oligonucleitides

550
Q

Western blotting

A
  • used to identify target polypeptide from saple
  • target proteins undergo gel electrophoresis
  • separated proteins are probed with specific antibody of interest
  • then radioactive marker is bound to first antibody
551
Q

Metalloproteinases

A

Zn-containing enzymes that degrade extracellular matrix.

- participate in tissue remodeling and in tumor invasion

552
Q

Angioedema

A
  • rare but serious effect of ACE inhibitors
  • involves swelling of tongue, lips, or eye lipds and pts may experience difficulty breathing
  • due to increased bradykinin levels
553
Q

ACE inhibitors are contraindicated in which patients?

A
  • In patients with peripheral vascular disease or atherosclerortic CV disease as they can develop ACUTE RENAL FAILURE
  • patients with renal disease dependent on ACE constriction to maintain renal failure and ACE inhibitors would reduce renal perfusion
554
Q

Causes of “LEFT” shift of oxygen - disassociation curve

A
  • indicates increased oxygen- hemoglobin affinity (“L= LUNGS”)
  • increased pH
  • decreased CO2, temperature and 2,3 DPG
555
Q

Causes of “RIGHT” shift of oxygen - disassociation curve

A
  • indicates decreased oxygen-hemoglobin affinity
  • decreased pH
  • increased CO2, temperature and 2,3 DPG
556
Q

Side effects of topic preparations of alpha-adrenergic agonists

A
  • Alpha adrenergics can cause vasocontriction of nasal mucosa vessels and are used nasal decongestants
  • Overuse can cause negative feedback resulting in decreassed NE synthesis
557
Q

Streptokinase

A
  • thrombolytic agents that acts by converting plaminogen to plasmin, which degrades fibrin
  • most common side effect is hemorrhage
  • foreign protein derived from Streptococci and can induce hypersensitivity reactions
558
Q

Regulation of iron absorption

A
  • regulated through hepcidin’s effect on absorption of dietary iron by intestinal epithelial cells and release of iron by macrophages
559
Q

Discuss iron absorption

A
  • After iron is absorbed by DMT-1, iron can take 1 of 2 pathways:
    1. may bind to ferritin (intracellular iron-binding protein) and remain stored within enterocyte. can be excreted into stool as enterocytes are sloughed off
    2. may enter circulation via ferroprotein, BL iron transporter of enterocyte. Free iron is transported by transferrin which becomes internalized after interacting with transferrin receptors on all cells
560
Q

B-thalassemia

A

HbA2 is elevated in B-thalassemia minor (trait) and B-thalassemia intermedia because B-globin chain underproduction causes decreased synthesis of HbA

561
Q

Hemoglobin components in adult blood

A 
HbA1 = (a2B2) - 97% of total Hb
HbA2 = (alpha2delta2) - 2.5% of total Hb
HbF = (alpha2gamma2) - < 1% of total Hb
562
Q

Hemophilia A

A
  • defect in coagulation factor VIII
  • NORMAL bleeding time (b/c bleeding is indication of platelet function)
  • prolonged PTT
563
Q

von Willebrand disease

A
  • vWF is a mediator for coagulation factor VIII and mediator of platelet aggregation
  • absence causes functional VIII deficiency and platelet deficiency
  • prolonged bleeding time (platelet function)
  • bleeding time
564
Q

Warfarin inhibits which

A
  • coagulants II, VII, IX, X

- anti-coagulants Protein C and Protein S

565
Q

K-ras

A
  • associated with cancers of bladder, lung, colon, pancreas and kidney
  • proto-oncogene (tumor promotor) resulting stimulation of cellular proliferation, inactivation of anti-oncogenes
566
Q

Proto-oncogenes

A
  • ras: bladder/colon/pancreas/kidney
  • ERB-B1: squamous cell of lung
  • ERB-B2: breast/ovarian cancer
  • TGFa: astrocytoma, hepatocellular carcinoma
  • sis: astrocytomas, osteosarcoma
  • abl: chronic myeloid leukemias
567
Q

Anti-oncogene

A
  • NFI
  • APC/B-catenin
  • DCC
  • p53
  • RB
  • WT-1
568
Q

Drugs that decrease progression of diabetic nephropathy

A
  • ACE inhibitors

- ARBs

569
Q

Age-related macular degeneration

A
  • leading cause of blindness in industrialized nations
  • involves degeneration of central retina
  • “dry AMD” - subretinal deposits leading to gradual loss of vision
  • “wet AMD” - more acute presentation - abnormal blood vessels with subretinal hemrrohage, gray subretinal membrane - due to increased VEGF
570
Q

Tx of wet AMD

A
  • due to increased vascular endothelial growth factors (VEGF) causing
  • treat with anti-VEGF
571
Q

Anti-inflammatory agents that do not inhibit platelet aggregation

A

Selective COX-2 inhibitors

**Platelets express mostly COX-1

572
Q

Drugs that inhibit thymidylate formation

A

Methrotrexate
5-FU

** Note that methotrexate can overcome by N5-formyl tetrahydrofolate (folinic acid, leucovorin)

573
Q

What metabolizes pro-carcinogens?

Hint: it’s in the liver

A

Converted by cytochrome p450 monooxygenase which is present in hepatocytes and ER of certain cells
- these enzymes convert the pro-oncogenes into active enzymes

574
Q

Which organ extracts blood more efficiently than any other organ?

A

Heart - thus venous blood is the most deoxygenated.

575
Q

HER2/neu oncogene

A
  • encodes 185kd transmemembrane protein that has intracellular tyrosine kinase activity
  • member of EGFR family
  • overexpression can lead to breast and ovarian cancers
576
Q

Statin side effect

A

Myopathy (leading to muscle pain)

- Statins commonly prescribed after MI as they reduce cholesterol and stabilize atheromatous plaques

577
Q

Sx of T- ALL

A
  • usually young child

- presents with mediastinal mass that can cause respiratory symtoms, dysphagia or superior vena cava syndrome

578
Q

Aldosterone excess

A
  • will canse hypertension, hypokalemia, metabolic alkalosis and depressed renin
579
Q

Aldosterone antagonists (e.g. eplerenone and spironolactone)

A
  • can cause hypoaldosterone (which can cause type IV renal tubular acidosis
  • can be used as medical therapy for Conn’s syndrome (adrenal mass)
580
Q

Aldosterone

A
  • secreted by adrenal glands
581
Q

Raltegavir

A
  • integrase inhibitor that disrupts ability of HIV to integrate genome into the host’s cells chromosome, thus preventing host cellular machinery from being used to synthesize HIV mRNA
582
Q

Patients with Sickle Cell Trait

A

HbA - 60%
HbS - 40%
** usually assymptomatic and have relative protection from malaria
- usually have normal peripheral smears and indices, reticulate counts and MCHC values
- may have RBCs that will sickle when sodium metabisulfite is added

583
Q

Vinicristine side effect

A
  • neurotoxicity (dose-limited) - results form failure of microtubule polymerization in neuronal axons
  • pulmonary fibrosis and flagellate skin discoloration
584
Q

Bleomycin side effect

A

Pulmonary fibrosis and flagellate skin discoloration

585
Q

Doxorubicin side effect

A

CHF

586
Q

Cycophosphamide side effect

A

hemorrhagic cystitis

587
Q

Transketolase

A
  • carry out non-oxidative reactions of HMP shunt
  • some cells don’t use oxidative phase to produce cytosolic NADPH but ALL cells can syntehsize fructose-6-phosphate using the non-oxidative reactions
588
Q

Giant cell arteritis

A
  • characterized by granulomatous inflammation of media, with fragmentation of internal elastic lamina of medium and small branches of carotid artery
  • most common in > 50 y.o paitent
  • jaw claudication is most important symptom
  • irreversible blindness due to ophthalmic artery occlusion is severe complication of GCA
  • Tx: immediate prednisone therapy
589
Q

Cherry hemangioma

A
  • small red cutaneous papules common in aging adults
  • don’t regress spontaneously and increase in number with age
  • micro: proliferation of capillaries and postcapillary venules in the papillary dermis
590
Q

Drug to prevent venous thrombosis in non-ambulatory patient or patients undergoing elective surgery

A

Heparin - increases the effect of naturally occuring anti-thrombrin III

591
Q

Ancathocytes

A

“spur ce;;”

- seen in abetalipoproteinemia

592
Q

Teardrop cells

A
  • seen in myelofibrosis.

- when bone marrow is replaced with fibrosis (or metastatic cancer), RBCs must squeeze through spaces

593
Q

Target cells

A 
"bulls eye" cells
- seen in "HALT"
HbC
Asplenia
Liver disease
Thalassemia
594
Q

Schistocytes

A 
- seen in DIC, TTP/HUS
traumatic hemolysis (e.g. metal heart valve prosthesis)
595
Q

DIC

A
  • complication of gram - sepsis, acute pancreatitis, and burn injury
  • gram negative spesis - activatio of coagulation cascade by bacterial endotoxins, causing formation of micro-emboli
596
Q

Lab values for DIC

A
  • fragmented RBCs (microangiopathic hemolytic anemia) and thrombocytopenia
  • prolongation of PT, PTT, and decreased fibrinogen, factor V, and factor VIII levels
597
Q

Most common cause of hypochromic, microcytic anemia

A

Iron deficiency

** most common cause of iron deficiency is blood loss

598
Q

Suspected etiology of iron deficiency in woman of childbearing age

A
  • Menstruation
599
Q

Suspected etiology of iron deficiency of blood loss in adult male or postmenopausal woman

A
  • Occult blood loss in GI tract
600
Q

Common cause of thrombocytopenia
(esp. in patients with ecchymoses, petechiae, and mucosal bleeding with signs and symptoms of TTP/HUS, pancytopenia, marrow failure, and splenomegaly

A

Autoimmune destruction of platelets

601
Q

ACE inhibitors on R-A-A- system

A
  • INCREASED RENIN (due to decreased renal perfusion)
  • INCREASED ANGIOTENSIN I, because renin converts angiotensinogen to angiotensin I
  • DECREASED ANGIOTENSIN II because ACE inhibitors prevent this conversion
  • DECREASED ALDOSTERONE because no angiotensin II to increase aldosterone secretion and induce vasoconstriction
  • INCREASED BRADYKININ - because less angiotensin II means less degradation of bradykinin

-

602
Q

Patient experiences parasthesias after receiving several units of blood. Serum calcium level is 7.2 mg/dL (hypocalcemic).
What’s the etiology?

A
  • Patients who receive more than one body blood volume (5-6 L) over 24 hrs may have elevated citrate levels (substance added to blood)
  • Citrate chelates calcium and magnesium and may reduce their plasma levels thus causing parasthesias
603
Q

Discuss carbon monoxide poisoning and methemoglobinemia

A
  • binds to hemoglobin with much higher affinity than oxygen, thus preventing oxygen from binding to hemoglobin
  • DOES NOT AFFECT OXYGEN SATURATION
  • DOES NOT CAUSE METHEMOGLOBINEMIA
604
Q

Dysplasia

A
  • REVERSIBLE change in epithelial cells
  • progress through low-grade dysplasia –> high-grade dysplasia/carcinoma in site –> invasive carcinoma
  • once breached basement membrane, no longer reversible
605
Q

Amlopidine

A
  • calcium channel blocker used to treat hypertension

side effect: flushing and peripheral edema

606
Q

Discuss Hemoglobin S in deoxygenated state

A
  • HbS aggregates in deoxygenated state
  • ## form fibrous strands that reduce RBC membrane flexibility and promote sickling
607
Q

Biphospoglycerate mutase

A
  • produced in large quantities in erythrocytes

- creates 2,3 BPG from 1,3 BPG

608
Q

Discuss 2,3 BPG and oxygen delivery to peripheral tissues

A
  • 2,3 BPG is produced in erythrocytes with enzyme biphosphoglycerate mutae
  • increased 2,3 BPG DECREASES affinity of Hb for oxygen thus allowing oxygen delivery to peripheral tissues

USMLE Step 1 (34 decks)

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