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Flashcards in Quick Associations 2 Deck (284):
1

A girl with Turner's syndrome --> high or low gonadotropin levels (LH & FSH) ?

will have high LH & FSH but streak ovaries and therefore need donor oocytes to try to become pregnant

2

Catalase +, Coagulase negative bacteria that cause endocarditis on prosthetic valves

Staph. epidermidis (treat with Vanc)

3

Fat cells that contain multiple fat droplets and numerous mitochondria per cell, seen in babies

Brown fat -- used for heat production! ((the ox phos and ETC in these cells is uncoupled so all energy produced is not made into ATP but instead dissipated as HEAT))

4

What protein is used in brown fat to uncouple the oxidative phos and ETC?

Thermogenin

5

Most carcinogens enter the body as pro-carcinogens and are converted to carcinogens by ________________

cytochrome P450 Monooxygenase enzymes

6

Why is someone on TPN who had a jejunal resection at increased risk for gallstones?

the lack of enteric stimulation --> decreased CCK secretion --> biliary stasis

7

________ in erythrocytes converts CO2 & water into bicarb

Carbonic anhydrase

8

temporal arteritis is characterized by what kind of inflammation

granulomatous of the media w/ fragmentation of the internal elastic lamina ((treat asap w/ steroids to prevent blindness!))

9

autosomal recessive disorder of tyrosine metabolism that results in a buildup of Homogentistic acid --which turns urine black after sitting out and attaches to collagen making the ears & nose appear blue/black

Alkaptonuria (defect of Homogentisate oxidase enzyme) --> in the tyrosine ---> Fumarate pathway

10

a defect in phenylalanine --> tyrosine pathway

PKU (phe hydorxylase defect)

11

What is the mechanism of methicillin resistance? (ex: MRSA)

the bacteria alter the penicillin binding proteins (makes them resistant to all beta - lactams)

12

What is the mechanism of resistance of Staph aureus (non MRSA) to beta lactams?

produces beta-lactamase ( an enzyme that degrades the beta lactam ring)

13

Acanthosis nigricans assoc with?

Insulin resistance (DM, Obesity, acromegaly) and rarely GI malignancies

14

Why are pregnant women or women on OCP's more likely to develop gallstones?

Estrogen -->increases cholesterol synth & secretion
Progesterone --> slows GB emptying & reduces bile acid secretion

15

CD3 is found on what cells?

T cells (needed for T cell receptors) --> anti CD3 antibody drugs can be used to inhibt T cells in acute rejection of kidneys, heart and liver transplants

16

older man who worked in a rubber/textile/ or leather plant for 30 years, now experiencing new onset gross hematuria, serum creatinine is normal

transitional cell carcinoma

17

HTN with low plasma renin activity and pt is feeling weakness / paresthesias

Primary hyperaldosteronism (normal Na levels due to "aldosterone escape", low K+ , and high bicarb)

hypokalemia = weakness & paresthesias

18

Side effects of SSRI's (sertraline, fluoxetine, citalopram, etc...)

Sexual dysfunction!

19

Pts with essential fructosuria cannot metabolize Fructose in the normal pathway (via Fructokinase) , but they can still put it into the glycolysis pathway via what enzyme?

Hexokinase

20

Pt with hx of hyperlipidemia has severe pain 40-60 minutes after eating, weight loss, pain not relieved by antacids

atherosclerosis of the mesenteric arteries --> they can't dilate in response to incrs blood demand after meals, leading to intestinal ischemia & pain

21

Activation of which enzyme is primarily responsible for the damage done in acute pancreatitis?

Trypsin (once activated, it activates other pancreatic enzymes and can auto-activate more trypsin)

22

What is the most common and 2nd most common form of cancer in children?

1- Leukemia
2- Brain neoplasms (Pilocytic astrocytoma is most common, Medulloblastomas are 2nd most)

23

Most common location for Pilocytic Astrocytoma

mostly in the Cerebellum -- looks like a cystic mass with a tumor nodule sticking off of it -- pretty good prognosis

24

most common malignant brain tumor in kids, only in the cerebellum (mostly vermis) --> solid with sheets of small round blue cells -- poor prognosis

Medulloblastoma

25

Anti-gliadin antibodies, loss of intestinal villi

Celiac dz

26

Aldolase B deficiency

causes life threatening hereditary fructose intolerance

27

Vitamin associated with maintaing specialized epithelium (acinar cells, etc...)

Vitamin A

28

Deficiency of this vitamin can cause infertility

Vitamin E

29

Treatment of acute Diphtheria infection

1- Diphtheria antitoxin (passive immunization)
2- Penicillin or Azithro
3- DPT vaccine

30

insomnia, agitation, dysphagia, headaches, coma after cave exploring

Rabies encephalitis

31

Stop codons

UAA, UGA, UAG

32

Type of diabetes associated with DKA

Type 1 (autoimmune)

33

Formula for Renal Plasma Flow (RPF)

Use the Clearance of PAH :

RPF = Urine PAH x Flow / Plasma PAH

34

Formula for Renal BLOOD Flow

RPF / (1 - Hematocrit)

35

Most fluids and solutes are reabsorbed in what part of the nephron?

PCT (fluid is absorbed isosmotically in the PCT --> no concentration or dilution occurs)

36

In a dehydrated state the kidney will concentrate the urine to a max of ___mOsm/L --- but it must put out at least .5 L / day to get rid of metabolic wastes (so will lead to death if you go to long without water)

1200 mOsm/L -- does this via ADH acting on the collecting duct to cause water conservation

37

The ________ of the nephron is impermeable to water

Ascending limb of the loop of Henle & the early distal tubule

38

Pt will 2 month history of weight loss, polydipsia, fruity urine

DKA

low pH
low bicarb -- anion gap metabolic acidosis
low PaCO2 -- resp alkalosis (compensatory)

39

A substance that constricts the efferent arteriole in the glomerulus will have what effect on GFR and Filtration Fraction?

-efferents constricted will INCREASE GFR and decrease RPF therefore -->INCRS FF

FF = GFR / RPF

ex: Angiotensin II would do this

40

ADH acts on what part of the nephron specifically ?

the medullary portion of the collecting duct (it increases the permeability to water by adding porins to the CD)

41

Where is Renin made and secreted and under what circumstances?

the JG apparatus, in response to decreased renal arteriole pressure & increased renal sympathetic discharge (**there are Beta 1 receptors on the JG apparatus!)

42

Mechanism of loop diuretics

inhibit Na-K-2Cl symporters in the thick ascending loop of Henle (essentially cause the excretion of these)

43

Pt presents hyperventilating (resp alkalosis) and then later enters into an anion gap metabolic acidosis

Aspirin overdose -- ASA directly stimulates the breathing center in the brain (low pCO2) and then hours later you get a metabolic acidosis due to the organic acid build up in the blood... (low bicarb)

44

Pt with a tear at the gastro-esophageal junction & history of alcoholism --> what kind of metabolic disturbance is going on?

Mallory-Weiss tears from vomiting --> loss of acid in vomit causes a metabolic alkalosis

45

This substance is filtered, secreted by the PCT, but not reabsorbed -- so the concentration of it rises as it goes throughout the nephron

PAH

46

Which part of the nephron is the primary site of K+ regulation?

the Cortical Collecting Duct

47

Aldosterone acts on the _______ to cause reabsorption of ____ & _____ and loss of ____ & ______

reabsorbs Na+ and water at the expense of K+ and H+

48

ADH (vasopressin) causes an increase in water reabsorption and _____ reabsorption from the PCT

Urea

49

This substance is filtered only, NO reabsorption or secretion

Inulin -- that's why it's used to measure GFR

50

The descending loop of Henle causes the urine to become ______

hyperosmotic (hypertonic) --> it is permeable to water but not solutes so as the loop moves down into the kidney medulla where the interstitium is more concentrated, water leaves the loop --> concentrating the urine

51

Selective proteinuria

Albumin loss without the loss of the 'bulkier' proteins like IgG and macroglobulins

(ex: Minimal change dz)

52

Filtration barrier in the glomerulus is made up of?

1- Fenestrated endothelium of arterioles
2- GBM
3- Epithelial cells (foot processes)

53

What is the main thing keeping Albumin from being filtered

The negative charge on the GBM and foot processes that repel the negatively charged Albumin ((not it's size, it could fit through the slits))

54

Massive proteinuria (> 3.5 gm/day), edema, hyperlipidemia

Nephrotic syndrome

55

Cystic dilations of the medullary collecting ducts in the kidney

Medullary sponge kidney --> pretty benign, cause unknown, can develop hematuria or kidney stones

(stones are # 1 complication )

56

#1 cause of Chronic Renal Failure

Diabetes

57

Hematuria, proteinuria, RBC casts and "hypercellular glomeruli" on microscopy

Post-strep glomerulonephritis

-will have high ASO titers and low C3
- subepithelial humps
- usually after throat or skin infection

58

Anti- GBM antibodies

Goodpastures syndrome --> renal failure, hemoptysis, crescents

59

Calcium oxalate crystals in the urine

Ethylene glycol ingestion (produces an anion gap metabolic acidosis)

60

Renal Clear cell carcinoma originates from what cell type

the PCT cells --> will appear golden/yellow grossly due to high fat content

61

When blood flow is compromised, what parts of the nephron are at most risk for low perfusion

Structures out in the medulla -> the PCT & thick ascending loop of Henle

62

Muddy brown casts in the urine

Acute Tubular Necrosis

63

bilateral flank masses (cysts) at birth or early in life, enlarged kidneys at birth

ARPKD

64

microscopic cysts in kidneys at birth that grow over time, can be clinically silent, some pts have flank pain, HTN, hematuria, renal failure slowly progresses over 10-20 yrs, also at risk for cerebral aneurysms

ADPKD

65

Most common nephrotic in white adults, assoc w/ Solid Tumors, hepatitis B or C, SLE or drugs, shows "spike & dome" appearance & capillary wall thickening

Membranous glomerulopathy -- due to immune complex deposition (IgG)

66

Multiple Myeloma signs & symptoms

Constipation (hypercalcemia)
Renal failure
Anemia (fatigue)
Bone pain (usually back & ribs)

-**Bence-Jones (light chains) & Tamm Horsfall proteins show up as eosinophilic casts (pink on H&E, not eosinophils the cells..)

67

Why do pts with nephrotic syndrome also get hyperlipidemia?

The liver tries to compensate for the massive loss of albumin but can't keep up so it ramps up production of other proteins too (including lipoproteins)

68

A baby born with no kidneys dies 12 hours after birth --> what is the cause of death?

Lung immaturity

-Potters syndrome from bilateral renal agenesis --> oligohydraminos --> no urine output, no lung development in utero

69

diffuse effacement of the foot processes of the glomerulus on EM

Minimal change dz --> treat with corticosteroids

70

linear deposits of IgG & C3 along the GBM

Goodpasture's --> a form of RPGN, will see crescents

71

IgA mediated vasculitis in children after a URI --> abdominal pain, joint pain, palpable purpura, hematuria

Henoch-Schonlein Purpura

72

Types of Rapidly Progressive Glomerulonephritis (all are crescentic)

Type 1 - anti-GBM ab's, (Goodpastures), linear deposits of IgG & C3
Type 2 - immune complex mediated, subepithelial humps (Post-strep glomerulonephritis, SLE, IgA nephropathy or Henoch Schonlein)
Type 3 - 'pauci immune' (no IgG or complement deposis), + ANCA (mostly Wegeners)

73

Patient with colon cancer and new endocarditis, what bacteria ?

Strep. bovis

74

Endocarditis that is culture-negative -- which bacteria?

Haemophilus
Actinobacillus
Cardiobacterium
Eikenella
Kingella

"HACEK"

75

subacute bacterial endocarditis after dental work

S. viridans

76

Sudden cardiac death right after an MI is most likely caused by _____________

an arrhythmia (most likely V. fib)

77

Trousseau syndrome

migratory thrombophlebitis seen in patients with disseminated cancer (tumor releases pro-coagulants)

78

autosomal recessive long QT syndrome that is associated with neurosensory hearing loss - can lead to sudden death

Jervell & Lange-Nielsen syndrome -- problem with the K+ channels in the ear and cardiac muscle

79

chest pain on exertion relieved by rest, how much of the artery is blocked by plaque?

at least 70% -- stable angina happens when a vessel is > 70% clogged

80

Pt in a MVA with blunt trauma develops sever hypotension, JVD and his systolic BP falls 15 mmHg with each inspiration ...

pulsus paradoxus, hypotension and JVD suggest something constricting the heart --> after a MVA, you should consider cardiac tamponade

81

young child with migratory arthritis 3 weeks after an untreated sore throat --> IF she were to die, what would be the most likely cause?

Myocarditis -- the cause of death if you die from Acute Rheumatic Fever

- assoc with Aschoff bodies & "Anitschkow" cells (caterpillar cells)

82

yellow-brown pigment found in cells of older people, made from lipid peroxidation & free radical injury (common in the heart & liver of aging or sick / malnourished people)

Lipofuscin

83

STEMI in leads II, III & aVF --> which artery is probably occluded?

Right Coronary Artery --- the posteroinferior wall of the heart is usually supplied by the posterior descending branch of the RCA

84

STEMI in leads V1 - V4 , what artery is most likely occluded?

the LAD (anterior /septal region)

85

STEMI in leads V5 - V6 & 1 & aVL, what artery occluded?

lateral circumflex (branch of the LCA)

86

STEMI in V1-V6 and 1 & aVL , which artery?

Left coronary artery (before it branches)

87

In older people (70's & older), what predisposes the aortic valve to calcifications?

Necrosis of the tissue / cell injury (injury -> necrosis -> dystrophic calcifications)

88

congenital defect in the lower interatrial septum is associated mostly with what congenital dz?

Down Syndrome (endocardial suchion defects are common ((like ostium primum ASD))

89

cardiac anomalies associated with DiGeorge syndrome

Tetrology of Fallot or aortic arch defects

90

Hypertrophic cardiomyopathy is associated with a mutation affecting what structures?

the sarcomere proteins (myosin, troponin, etc...)

91

Dilated cardiomyopathies are assoc with mutations affecting what structures?

cytoskeletal proteins

92

most common cause of mitral stenosis

rheumatic heart dz ((usually starts as regurgitation and progresses to stenosis))

93

Kussmaul's sign indicates

constrictive pericarditis (( an increase in JVP upon inspiration ))

94

vegetations on BOTH sides of a valve indicate

non-infectious "Libbman- Sacks" endocarditis --> assoc with Lupus

95

Painless, hemorrhagic macules on the soles & palms

Janeway lesions (septic microemboli assoc with bacterial endocarditis)

96

Painful red lesions on fingers & toes

Osler's nodes (Osler = Ouch!) -- also associated with bac endocarditis

97

chest pain that is reversible, relieved by nitro, and occurs at rest

UNstable angina (ST depression to just endocardial ischemia)

98

Angina that involves coronary vasospasm

Prinzmetal's angina (ST elevation due to transmural ischemia)

99

mainstay of treatment fot heart failure

ACE inhibitors

100

heart defect associated with fetal alcohol syndrome

VSD

101

heart defect assoc with maternal diabetes

Transposition of the great arteries

102

caused by molecular mimicry of the bacterial M protein (which mimics a lot of human proteins)

Acute rheumatic fever

103

low voltage EKG with diminished QRS amplitude

Restrictive cardiomyopathy

104

most common adult primary heart tumor

Myxoma , "pedunculated mass" in the left atrium , benign

105

most common childhood primary heart tumor

Rhabdomyoma, benign hamartoma, assoc with Tuberous Sclerosis

106

prognosis of colorectal carcinoma is mainky determined by ___

stage (not grade)

107

benign hypopigmented patches caused by the fungus Malassezia furfur

Tinea versicolor -- KOH prep reveals "spaghetti & meatballs" appearance on light microscopy

108

Homeobox genes (Hox genes) code for what kind of proteins ?

transcription factors -- play a role in morphogenesis

109

apical displacement of the tricuspid valve, atrialization of the right ventricle and huge right atrium

Ebstein's anomaly -- assoc with Lithium use

110

child w/ fever, dysphagia, drooling, anxious, dyspnea , inspiratory stridor

Epiglottitis --- could be prevented with the Haemophilis influenza type b vaccine

111

Hepatic encephalopathy will cause a decrease of _____ & ______ in the brain

alpha-ketoglutarate and glutamate , which inhibits the kreb cycle (glutamine accumulates causing astrocyte swelling)

112

Hand flapping in a pt with cirrhosis and testicular atrophy

asterixis

113

where are steroid hormones made (which organelle)?

Smooth ER

114

drug that inhibits the synthesis of guanine and is used for Hepatitis C and RSV

Ribavirin

115

defect in Fibroblast growth factor 3 (FGF3) will cause

achondroplastic dwarfism

116

secreted by parafollicular C-cells in the thyroid in response to high blood calcium

Calcitonin

117

what happens to c-peptide after it is cleaved from pro-insulin in the pancreatic B-cell golgi?

it is packaged in secretory granules and secreted in equimolor concentrations with insulin ((it is used to distinguish Type 1 from type 2 diabetes b/c it represents the amount of insulin you have))

118

In the migration of neural crest cells to the intestine, which segment will recieve innervation last in development?

the rectum ((the NCCs migrate caudally --> so the rectum is always the last to get them and is always involved if the baby has Hirschsprung's))

119

African american female with hilar adenopathy, pulmonary infiltrates and non caseating granulomas

Sarcoidosis --driven by TH1 cells, IL-2 & IFN-gamma (which stimulate more TH1 and macrophages)

120

drug of choice for treating trigeminal neuralgia (tic douloureux)

Carbamazepine (reduces Na+ channel recovery and therby reduces neuronal firing)

121

child w/ large tonsils, cannot isotype switch antibodies

Hyper IgM syndrome

122

Fungal infection strongly assoc with DKA

Mucormycosis

123

Most dangerous side effect of Amphotericin B

Nephrotoxicity --- decreases GFR and also directly toxic to tubules (can lead to hypokalemia and arrhythmias)-- also look out for hypomagnesemia

124

Side effects of ACE inhibitors

Cough -- most common
Angioedema -- life threatening*
Proteinuria
Taste changes
HypOtension
Fetal renal damage
Rash
incrs Renin
Low AG II
HypERkalemia

125

Type of bias that occurs when the investigator's decision is affected by prior knowledge of the exposure status

Observer bias

126

Post-strep glomerulonephritis is an example of a Type ______ hypersensitivity reaction

Type III --Immune complex mediated

127

Side effects of Mannitol (an osmotic diuretic)

Pulmonary edema, dehydration

-pulmonary edema is caused by the rapid rise in plasma volume due to the drug pulling water out of cells and reducing intracranial pressure

128

IV acyclovir can cause crystalline nephropathy -- but can be prevented by ____

adequate hydration (fluids!!)

129

Spironolactone can increase overall survival from CHF how??

antagonizes aldosterone and aldosterone is known to cause ventricular remodeling and cardiac fibrosis in CHF patients

130

Which population should receive reduced doses of renally cleared medications to prevent toxicity?

the elderly --- there is a decrease in renal clearance / function as we age (be aware that there is not always a concomiant increase in Creatinine in elderly b/c as their renal function is decreasing , so is their muscle mass...)

131

the loss of E-cadherin is associated with ______

cancer metastasis ---> E-cadherin helps cells stay together

132

what is Calcineurin?

a protein in T cells that is needed for the activation of IL-2 --> growth of T-cells

133

dz in which ceramide trihexoside accumulates in tissues--> leading to dark red rash btw umbilicus & knees (angiokeratomas), neuropathic pain & and renal failure

Fabry's disease

134

man with recurrent kidney stones, besides hydration, what drug can help prevent recurrances?

HCTZ --thiazide diuretics decrease renal Calcium excretion

135

Calcineurin (T-cell) inhibitors used to prevent graft rejection

Cyclosporine & Tacrolimus

136

drug of choice for acute CHF -- b/c they provide maximun diuresis in shortest amount of time

Loop diuretics

137

IBD that involves only the colon / rectum, has mucosal/submucosal inflammation, main symptom is bloody diarrhea

Ulcerative Colitis

138

Which vitamin has been shown to improve the outcome of measles?

Vitamin A

139

Bacteria that substitute D-lactate for D-alanine in the formation of the cell wall are resistant to ______

Vancomycin

140

Type II error (Beta)

saying that there is no difference when there really is one (Beta)

141

Power of a study

the probability of seeing the difference when one truly exists (Power = 1 - Beta)

142

findings of a subdural hematoma and bilateral retinal hemorrhages in a baby

Shaken baby syndrome --> report to state protective agency

143

damage to this nerve will result in the loss of the 'knee jerk' patellar reflex

Femoral (L2-4)

144

symptoms of a right sided colon mass

usually an exophytic mass --> anemia (from bleeding), fatigue/ pallor, anorexia, malaise, weight loss...

145

symptoms of a left sided colon mass

tend to infiltrate the wall --> obstruction, tenesmus, ribbon stools, N/V/Constipation..

146

Risk factors for Osteoporosis

Smoking
Menopause
Corticosteroids
Physical Inactivity
Caucasians
Low body weight / BMI
Alcohol use

147

The receptors for PTH are on _________

Osteoblasts --> which then turn on RANK --> activates osteoclasts to start resorbing bone and increasing serum calcium

148

patient who works around goat wool and develops hemoptysis, wide mediastinum..

Pulmonary Anthrax

149

gram + rods that form "medusa head" colonies and posses an antiphagocytic D-glutamate capsule

Bacillus anthracis

150

gram negative bacteria that have an IgA protease which helps them get into mucosal tissues

Neisseria gon. and menin.

151

Name 2 ANTI-inflammatory cytokines

TGF-beta and IL-10

152

What test differentiates Strep from Staph?

Catalase test (Strep = catalase negative) , (Staph = catalase +)

153

What is the ONLY coagulase + Staph species?

S. aureus

154

Pt has hypothyroidism (w/ low T4 and low TSH) -- is this Primary or Secondary hypothyroidism?

Secondary --> problem is the pituitary

155

pt with schizophrenia -- treatment ?

Olanzapine (or another antipsychotic)

156

Pt gets exposed to Radioactive iodide --> what can you give them to protect their thyroid ?

K+ iodide

157

Senile osteoporosis --> high or low turnover?

Low turnover
- Osteoblasts low
-Osteoclasts normal or high

158

Post-menopausal osteoporosis --> high or low turnover?

High turnover
- osteoblasts & clasts are high
-bone is lost b/c formation can't keep up with absorption

159

What kind of receptors are the T3 & T4 hormone receptors?

nuclear / retinoid X binding to DNA

160

glycine & GABA are _____ neurotransmitters & glutamate is a _________ nt

inhibitory , excitatory

161

Diseases caused by S. aureus preformed EXOTOXIN

1- TSS
2- SSSS
3- Gastroenteritis (food poisoning)

162

1 gram protein or carbohydrate = ___ calories
1 gram fat = ___calories
1 gram ethanol = ___ calories

1 gram protein or carbs = 4 cals
1 gram fat = 9 cals
1 gram ethanol = 7 cals

163

_____________ has a very low infectious dose --> as few as 10 organisms can cause disease (although usually more)

Shigella

-other organisms that can infect at low #'s:
- Campylobacter (500 organisms)
- E. histolytica & Giardia (1 organism)

164

infant w/ vomiting, lethargy, jaundice shortly after beginning breastfeeding

Classic galactosemia (most common form)

- lack of galactose-1-phos uridyl transferase

165

hormone made by cells in the duodenum that increases bicarb secretion from the pancreas

Secretin

-stimulated by acidity (HCL)

166

pathogenic mechanism of Shigella

mucosal invasion

- invades M cells in peyers patches and causes bloody diarrhea
-also releases Shiga toxin --inhibits protein synthesis

167

the best way to determine if a certain gene is being expressed in tissue is to analyze the presence of ______________ by _______ blot

mRNA by Northern blot

(mRNA --> protein) = being expressed

168

What is the best way to destroy Hepatitis A on contaminated fomites or in water?

Boiling for 1 minute

(Hep A is fecal-oral) -- can also use UV light, bleach or chlorination

169

_______ is only capable of infecting when encapsulated with HBsAG

hepatitis D (delta agent)

170

a woman with HepB is most likely to pass it to her baby if she is _______ positive

HBeAg (the envelope)

-HBeAg is a marker of increased infectivity and replication

171

Which enzyme in the TCA cycle has Thiamine as a cofactor?

alpha-ketoglutarate DH

(a-kg to succinyl coA)

172

enzyme that is SPECIFIC to the liver that breaks down glycerol

Glycerol kinase

173

alpha- glucosidase deficiency in an infant

Pompe's disease

-glycogen storage dz type 2

- cardiomegaly**
- hepatomegaly
- macroglossia, severe MR
- glycogen accumulates in lysosomes

174

glycogen phosphorylase deficiency in an infant

McArdle's disease

-glycogen storage dz type V

- muscle glycogen phosphorylase defic
- weakness / fatigue when exercise **
- blood lactate levels don't rise after exercise

175

deficiency in Glucose - 6 phosphatase in an infant

von Gierke's disease

- most common glycogen storage dz (Type 1)
- hepatomegaly
- fasting hypoglycemia (seizures)
- hyperuricemia
- hyperlipidemia

176

glycogen debranching enzyme deficiency

Cori's or "forbes' disease

- glycogen storage dz type III

- hepatomegaly
- growth retardation**
- hypoglycemia
- hyperlipidemia
- muscle weakness

177

a tumor in the 3rd part of the duodenum is at risk for penetrating into what vessel?

the SMA

178

pt with severe hypoglycemia but high hepatic intracellular NADH ---> where is the liver getting it's energy?

hypoglycemia in the setting of high NADH = the liver is getting energy from something other than glucose

-Ethanol is an example (alcoholics)

179

What CANNOT use ketone bodies for energy?

RBC's and Hepatocytes

180

LOCAL defense against candida is done by _____ and SYSTEMIC defense is done by ______

Local --> T-cells (ie: HIV pts usually have localized candida)

Systemic --> Neutrophils (neutropenic pts can have systemic)

181

a bleeding ulcer at the posterior duodenal bulb is most likely to penetrate what artery?

gastroduodenal artery

182

delta - aminolevulinic acid is elevated in patients with _______

lead poisoning

(and Pyridoxal phosphate is a necessary cofactor for it so it also elevated)

183

technitium -99 scan shows the presence of __________

ectopic gastric epithelium

(will help identify Meckel's diverticulum which often has ectopic gastric mucosa)

184

which part of the intestine is the main site of Lipid absorption?

JEJUNUM

185

psoriasis can be associated with

deforming joint disease (psoriatic arthritis)

186

BRAF mutation (w/ Valine 600 replaced by glutamate) is seen in patients with ______

Melanoma

187

a chronic inflammatory skin condition associated w/ several environmental factors (foods, irritants..), intensely pruritic & associated with asthma & allergic rhinitis

Atopic Dermatitis (aka "Eczema')

-cannot be diagnosed unless itchy!

-Eczema + Allergic Rhinitis + Asthma = the "Allergic Triad"

188

histological changes seen in Urticaria

dermal edema + dilated lymphatics (no changes in epidermis)

189

most common site for bullae of Pemphigus vulgaris

Mouth / mucosal surfaces

-Bullous pemphigoid spares the mouth/ mucosa

190

axillary lymph node dissection after mastectomy predisposes you to chronic lymphedema & development of ________

angiosarcoma (Stewart-Treves syndrome)

191

What is Vitiligo?

autoimmune destruction of melanocytes

192

Most common sites of fracture in osteoporosis

1- vertebral crush fractures
2-femoral neck
3- Colle's fracture (distal radius)

193

Achondroplasia inheritance pattern

Autosomal dominant activating mutation of FGFR3 --(but most are sporadic mutations)

194

increased Alk phos in bone disease (ie: Paget's) indicates_______

increased osteoblast activity

195

soap bubble tumor of the epiphysis

Giant Cell tumor (osteoclastoma) -- benign

196

11:22 translocation

Ewing's Sarcoma -- small round blue cell tumors, in diaphysis of long bones, malignant

197

autoantibodies to ribonucleoproteins (SS-A and SS-B)

Sjogren's syndrome

198

Anti-Jo-1 antibodies

Dermatomyositis / Polymyositis

199

drugs highly associated w/ SJS

Bactrim, Dilantin

200

'sawtooth' infiltrate of lymphocytes at the dermal/epidermal junction , this dz also highly assoc w/ Hep C

Lichen planus

201

S-100 tumor marker

Melanoma

202

drugs that end in '-dronate'

block osteoclasts

(bisphosphonates used for Paget's, osteoporosis, hypercalcemia)

203

unique toxicity side effect of bisphosphonates

osteonecrosis of the jaw, and esophagitis

204

landmark for doing a pudendal nerve block

ischial spines

205

What can be used to treat the side effects of Myasthenia gravis drugs (like pyridostigmine)?

-diarrhea, nausea, sweating, cramping...

Pyridostigmine is a cholinesterase inhibitor (leading to increased Ach effects throughout the body)


-you can use an antimuscarinic agent like Scopolamine --> it treats only the side effects & doesn't counter -stigmine's effects on muscle b/c the muscle receptors are nicotinic cholinergic and Scopolomine blocks the muscarinic receptors only....

206

what nerve supplies the gluteus medius & minimus?

Superior gluteal n

207

test you must perform on your patient before starting them on any TNF-alpha inhibitors

PPD skin test (check for latent TB that could become reactivated)

-TNF-a inhibitors:
-etanercept, infliximab & adalimumab

208

what is the primary abnormality in Paget's dz of the bone?

Osteoclasts-- bone resorption

209

inheritance pattern of Osteogenesis imperfecta?

Autosomal dominant

210

plasma protein that binds free Hb to prevent it's renal excretion

Haptoglobin

-decrs in hemolytic anemias

211

young african american child with bilateral swollen & painful hands & feet

Dactylitis (a result of of sickle cell disease seen in young children)

212

Hydralazine & Procainamide are metabolized in the liver by________

phase II - N acetylation

(slow-acetylators develop Lupus like syndrome more often)

213

anti-centromere antibodies are specific for

CREST syndrome

- Scl-70 AB's are specific for systemic Scleroderma

214

drugs that are more __________ are preferentially processed in the liver (rather than kidney)

Lipophilic / good CNS penetration / high Vd

215

infant with testes but underdeveloped external male genetalia...which enzyme is deficient?

5-alpha-reductase

(can't convert testosterone ---> DHT)

216

man with tinnitus & vertigo is found to have bilateral acoustic schwannomas ---> a mutation on chromosome ____ is likely

Auto. dom. mutation of NF-2 tumor suppressor gene on chromosome 22 (codes for 'merlin' protein)

-bilateral acoustic schwannomas are classic in NF-2 (Central neurofibromatosis)

217

renal cysts, hemangioblastomas of the cerebellum, bilateral renal cell carcinoma...

VHL -- mutation on chromosome 3

218

prolactin is under tonic suppression by ______ from the ________

Dopamine from the hypothalamus

219

what is the best initial treatment for extreme hypoglycemia

IM shot of glucagon in an emergency

(IV dextrose once you get to hospital)

220

drug for isolated systolic HTN with main side effect of peripheral edema (ankle swelling) and flushing

Amlodipine

221

what does the mutation in CF actually do to the CFTR protein?

the Phe deletion causes abnormal post-translational processing so it never even gets to the cell surface -- it gets degraded

222

Symptoms of Vitamin E deficiency

degeneration of dorsal columns, peripheral nerves, cerebellar tracts..

-mimics Friedrich ataxia

223

long-branched lipids that contain about 90 carbons--- in a bacterial cell

Mycolic acid in Mycobacterium (what makes them "acid fast")

224

rupture of a berry aneurysm causes a ___________ hemorrhage

subarachnoid hemorrhage

225

after starting this HTN drug, you can expect the patient's serum creatinine to increase by up to 30%

ACE inhibitors -- b/c they prevent AgII from constricting the efferent arteriole, therby decreasing GFR

226

serotonin releasing neurons are ONLY found in the ____________

Raphe nuclei of the brainstem (axons then project all over the CNS releasing Serotonin)

227

urine draining from the umbillicus of a neonate suggests persistant __________

patent urachus / allantois remnant

228

meconium draining from the umbillicus of a neonate indicates persistant ________

yolk sac / vitelline duct

229

Apolipoprotein E4 is associated with ______

late onset Alzheimer's

230

mood stabilizers

Lithium, Valproate, Carbamazepine

231

the shortest acting benzodiazepine -- to prevent excessive fatigue

Triazolam or alprazolam

232

failure of neural crest cells to migrate to the truncal and bulbar ridges of the truncus arteriosus and the bulbus cordis can lead to ____, _____ or _______

-Tetrology of Fallot
-Truncus Arteriosus
- Transposition of the Great Vessels

233

too rapid correction of hyponatremia can cause _________

osmotic demyelination syndrome (central pontine myelinolysis) --> spastic quadriplegia

234

if the sup. laryngeal nerve is damaged in thyroid surgery --> what muscle will lose innervation?

Cricothyroid muscle

(recurrent laryngeal n. supplies all the other larynx muscles)

235

drugs that are associated with fat redistribution from extremities to trunk

HIV protease inhibitors and long-term glucocorticoid use

236

a common complication of venous stasis/ varicose veins

venous stasis ulcers (usually on the medial malleolus)

237

a woman with significantly increased amniotic fluid --> baby is at risk for _____

anencephaly or GI obstruction (decrs fetal swallowing)

238

elevated acetylcholinesterase and AFP in amniotic fluid __________

neural tube defects

239

______ is believed to increase Insulin resistance in overweight people

increased Free fatty acids & Trigs

240

serous fluid collects within the tunica vaginalis

Hydrocele

241

most common cause of fetal hydronephrosis

inadequate recanalization of the ureteropelvic junction (junction of kidney & ureter)

242

this vitamin should NOT be taken by pt's on Levodopa b/c it decreases it's effectiveness

B6

243

drugs that can improve both the + and - symptoms of Schizophrenia

Second gen (Atypical) antipsychotics

-it's not typical for "OLd CLOsets to QUietly RISPer from A to Z"

244

what is the RLS in the conversion of Acyclovir to it's active triphosphate form

monophosphorylation by the viral Thymidine Kinase

245

best area on body to measure to get an idea of Insulin resistance

Waist-to-hip ratio (helps determine visceral fat)

246

mechanism of Mifepristone

anti-progestin drug (5X stronger affinity for progesterone receptor than progesterone)

247

mechanism of Phentolamine

alpha blocker

248

Which anti-fungal drugs act against the cell membrane (ergosterol)?

-Amphotericin B
-Nystatin
-Azoles

249

inheritance pattern of G6PD deficiency

X-linked recessive

250

FTA-ABS test is specific for _________

Syphilis

251

Polycythemia vera is associated with a mutation in ______________

Jak 2 - NON receptor tyrosine kinase

252

pt with sickle cell & gross hematuria

renal papillary necrosis

253

paralysis of upward gaze (parinaud syndrome) is characteristic of a _______________ lesion

dorsal midbrain around the pineal gland

254

this drug act to prolong both the PT & PTT time

FondaparinuX

-direct factor Xa inhibitor

(remember b/c factor X is the intersection of the intrinsic & extrinsic pathways so this drug will prolong BOTH PT & PTT)

255

best diet alteration for diabetes improvement

low-calorie (better than low carb)

256

most important risk factor for gastric adenocarcinoma

diet (smoked foods, nitrosamines), blood type A, H. pylori or autoimmune gastritis

(NOT SMOKING)

257

when you have a mitochondrial disease, why are there varying presentations?

Heteroplasmy :a mixture of more than one type of an organellar genome within a cell or individual.

258

pt with MR, night blindness, chronic diarrhea, weakness, ataxia, steatorrhea...

Abetalipoproteinemia

-lack of ApoB-100 & B-48 = can't absorb fat or fat soluble vitamins and all the consequences from that

259

Mechanism of Macrolide antibiotics
(ie: Erythromycin)

blocks translocation of the amino acid chain on the 50s subunit rRNA ("macroSLIDES")

260

pathogenesis of sickle cell anemia

HbS point mutation in Beta chain (glutamic acid --> valine), low O2 state or dehydration precipitates sickling --the deoxy Hb polymerizes to make sickle shape)

261

what is the function of the poly-A tail

protects the mRNA strand until it gets out of the cell (gets degraded from the poly-A tail and up)

262

function of peptidyl transferase

making the covalent peptide bonds btw amino acids on a growing peptide

263

causes your body to absorb too much iron from the food you eat

Hereditary hemochromatosis

264

drug therapy that you can use for Hepatic encephalopathy

Lactulose

-b/c it renders the ammonia inabsorbable by converting it to ammonium (NH4), and increase transit of bowel content through the gut.

265

ligament that attaches to the cervix and then goes posteriorly

Uterosacral ligament

266

effect of Cushing's ACTH on the adrenal cortex

hyperplasia

267

which structure assists in producing new bone after a fracture (ex: clavicle fx)

The periosteum

-contains the progenitor cells for osteoblasts, chondroblasts and fibroblasts

268

"Hot T-Bone stEAk"

IL-1 --- fever
IL-2 -- stimulates T cells
IL-3 -- stimulates bone marrow
IL-4 -- stimulates IgE
IL-5 -- stimulates IgA

IL-8 -- recruits neutrophils (clean up on 'aisle 8')

269

What is the most likely explanation for prolonged, ABX resistant colonization of a CF patient w/ Pseudomonas

the Pyocyanin toxin made by Pseudomonas...

270

virulence factor of N. gonorrhea

pili, opa proteins

271

function of Neuraminidase inhibitors

block the function of the viral neuraminidase protein, thus preventing the virus from reproducing by budding from the host cell.

272

which interleukin is also called 'osteoclast activating factor' and is involved in bone resorption

IL-1

273

Bence-Jones proteins

multiple myeloma

274

most grave effect of Metformin overdose

Lactic acidosis

275

which tissue proliferates in a hydatidaform mole?

trophoblastic tissue (what would have become the placenta)

276

What is the most commonly used drug for infertility?

(also used for PCOS)

Clomiphene

(SERM) that increases production of gonadotropins by inhibiting negative feedback on the hypothalamus.

277

True or False -- regular menses means regular ovulation

FALSE

-women undergoing anovulation still have (more or less) regular periods. In general, patients only notice that there is a problem once they have started trying to conceive.

278

Effects of a Beta-2 agonist

Smooth muscle relaxation (bronchial dilation, uterine dilation, relaxes GI tract & relaxes detrusor)

279

child dies from mycobacterial infection, had brother who also died from this... what is missing?

INF-gamma receptors (can't call macrophages)

280

mechanism of Cisplatin

crosslinks DNS

(very effective at treating testicular CA)

281

child dies from mycobacterial infection, had brother who also died from this... what is missing?

INF-gamma receptors (can't call macrophages)

281

child dies from mycobacterial infection, had brother who also died from this... what is missing?

INF-gamma receptors (can't call macrophages)

282

mechanism of Cisplatin

crosslinks DNS

(very effective at treating testicular CA)

282

mechanism of Cisplatin

crosslinks DNS

(very effective at treating testicular CA)