FA, Rx, UW

Question 1

Chromosome 3

Answer 1

VHL, Renal Cell Carcinoma

Question 2

Chromosome 4

Answer 2

ADPKD (PDK2), achondroplasia, Huntignton

Question 3

Chromosome 5

Answer 3

Cri-du-chat syndrome, FAP

Question 4

Chromosome 6

Answer 4

Hemochromatosis (HFE)

Question 5

Chromosome 7

Answer 5

Williams syndrome, CF

Question 6

Chromosome 9

Answer 6

Friedreich ataxia

Question 7

Chromosome 11

Answer 7

Wilms tumor, Beta-globin gene defects (e.g. Sickle cell)

Question 8

Chromosome 13

Answer 8

Patau syndrome, Wilson disease, RB, BRCA2

Question 9

Chromosome 15

Answer 9

Prader-Willi, Angelman, Marfan

Question 10

Chromosome 16

Answer 10

ADPKD (1), alpha-globin defects

Question 11

Chromosome 17

Answer 11

NF Type 1, BRCA1, p53

Question 12

Chromosome 18

Answer 12

Edwards Syndrome

Question 13

Chromosome 21

Answer 13

Down syndrome

Question 14

Chromosome 22

Answer 14

NF Type 2, DiGeorge Syndrome (22q11)

Question 15

What is Hartnup disease?

Answer 15

An autosomal recessive deficiency of Tryptophan; results in pellagra-like symptoms.

Question 16

Nondisjunction that produces trisomies occurs specifically at which stage of Meiosis 1/2?

Answer 16

Anaphase

Question 17

Which cancer is associated with t(8;14)?

Answer 17

Burkitt lymphoma (c-myc activation)

Question 18

Which cancer is associated with t(9;22)?

Answer 18

CML(BCR-ABL hybrid), ALL (less common, poor prognostic factor)

Question 19

Which cancer is associated with t(11;14)?

Answer 19

Mantle cell lymphoma (cyclin D1 activation)

Question 20

Which cancer is associated with t(14;18)?

Answer 20

Follicular lymphoma (BCL-2 activation)

Question 21

Which cancer is associated with t(15;17)?

Answer 21

APL (M3 type of AML)

Question 22

Which type of DNA repair mechanism is impaired in xeroderma pigmentosum?

Answer 22

Nucleotide excision repair

Question 23

Goodpasture’s Syndrome has autoantibodies against what?

Answer 23

Anti-glomerular basement membrane (Type IV collagen)

Question 24

What type of DNA repair mechanism is defective in Lynch Syndrome (HNPCC)

Answer 24

Mismatch repair

Question 25

Fragile X syndrome is which tirnucleotide repeat, and is characterized by what phenomenon?

Answer 25

CGG repeat; hypermethylation of the FMR1 gene resulting in decreased expression

Question 26

Which conditions result in a microcytic anemia?

Answer 26

Microcytic = MCV < 80fL - SALTI - Sideroblastic - Anemia of chronic disease - Lead poisoning - Thalassemias - Iron deficiency (late)

Question 27

Where does collagen synthesis, hydroxylation, and glycosylation take place?

Answer 27

In the Rough ER

Question 28

Where does collagen triple helix formation take place?

Answer 28

In the rough ER (hydrogen and disulfide bonds)

Question 29

Where does cleavage of the C- and N- terminals of collagen take place?

Answer 29

In the extracellular space --> forms tropocollagen

Question 30

A patient with increased lactic acid levels in the setting of acute mesenteric ischemia, most likely has decreased activity in which enzyme?

Answer 30

Pyruvate dehydrogenase - Someone with mesenteric ischemia has inadequate O2 delivery to the intestinal tissues, which means that the accumulation of NADH under hypoxic conditions inhibits pyruvate dehydrogenase.

Question 31

Which enzymes in glycolysis and the TCA produce NADH from NAD?

Answer 31

The dehydrogenases: - Pyruvate dehydrogenase - Isocitrate dehydrogenase - Alpha ketoglutarate dehydrognase

Question 32

Which cofactor is needed for transamination and decarboxylation reactions?

Answer 32

B6 (pyridoxine)

Question 33

An inability to transport ornithine into the mitochondria indicates what disorder?

Answer 33

Ornithine translocase deficiency, which precipitates a defect in the hepatic urea cycle. Tx: protein restriction

Question 34

Biotin is a cofactor for which enzymes?

Answer 34

The carboxylases: - Pyruvate carboxylase (Pyr-->OAA) - Acetyl-CoA Carboxylase (Acetyl-CoA--> Malonyl CoA) - Propionyl-CoA carboxylase (Propionyl-CoA-->Methylmalonyl-CoA)

Question 35

Of the non-glucose monosaccharides (mannose, fructose, galactose), which one bypasses phosphofructokinase?

Answer 35

Fructose

Question 36

What are homeobox genes?

Answer 36

Homeobox (Hox) genes encode DNA-binding transcription factors. Mutations in Hox genes cause the genes for different appendages to be transcribed in the wrong places (because the Hox genes are expressed at the wrong time/place).

Question 37

How does carbon monoxide (CO) impact hemoglobin?

Answer 37

CO competitively binds iron present in heme proteins. It has no impact on the partial pressure of O2.

Question 38

Which type of factor has Zinc-finger domains?

Answer 38

Intracellular receptors (e.g. steroids, thyroid hormone, fat-soluble vitamin receptors)

Question 39

Infusion of hemin (heme) for treatment of AIP, causes downregulation of what enzyme?

Answer 39

ALAS (aminolevulinate synthase) - the rate-limiting enzyme in the heme synthesis pathway

Question 40

True or False? Patients with aldolase B deficiency can tolerate sucrose?

Answer 40

False. Sucrose is composed of glucose and fructose (which can't be broken down in patients with aldolase B deficiency).

Question 41

How can fibrates cause cholecystitis?

Answer 41

Fibrates inhibit cholesterol 7alpha-hydroxylase which is the rate-limiting enzyme in the synthesis of bile acids. Without bile acids, decreased cholesterol solubility in bile favors cholesterol stone formation.

Question 42

How do the shapes of the hemoglobin and myoglobin dissociation curves differ?

Answer 42

The Hg curve is sigmoid in shape (due to positive cooperativity with O2 binding); the myoglobin oxygen dissociation curve is hyperbolic, because it only has a single heme group, and doesn't have heme-heme interactions/facilitation.

Question 43

Which findings are seen in Fabry disease?

Answer 43

X-linked Recessive Early: 1. Hypohidrosis, Angiokeratomas, Peripheral neuropathy Late: Renal Failure, LVH FABRY ACC - Febrile episodes (hypohidrosis) - Angiokeratomas - Burning pain (peripheral neuropathy) - Renal failure - Young death - Alpha-galactosidase A (deficient enzyme) - Cardiovascular disease - Ceramide trihexosidase (buildup product)

Question 44

Which findings are seen in Tay-Sachs disease?

Answer 44

"Tay's not heppy about developing a crush on his OG eX Amanda" - Not Heppy = NO hepatosplenomegaly - Developmental delay - CRush = Cherry Red spot - O = onion skinned lysosomes - G = GM2 ganglioside - X Amanda - HeXosaminidase A

Question 45

Which findings are seen in Krabbe disease?

Answer 45

"GOD, you're so Kabbe" - G = galactocerebrosidase/globoid cells - O = Optic atrophy - D = developmental delay

Question 46

Which findings are seen in Niemann-Pick disease?

Answer 46

"Niemann-picked up his heppy, neurotic father from central spot station" - Hepatosplenomegaly - Foam cells - Neurodegeneration - Cherry-red spot - Sphingomyelin/ase

Question 47

Which findings are seen in Hurler syndrome?

Answer 47

- Corneal cLouding (HurLer) - GargoLylism - HSM - alpha-L-iduonidase deficiency

Question 48

Which findings are seen in Hunter syndrome?

Answer 48

- NO corneal clouding (there is no "L" in "Hunter") - Aggressive behavior (Hunters are aggressive) - Iduronate sulfatase deficiency

Question 49

Which congenital disorder of predisposes patients to E. coli sepsis?

Answer 49

Classic galactosemia (deficiency in galactose-1-phophate uridyltransferase). Presents with failure to thrive(soon after the onset of infant feeding, within days), jaundice, Hepatomegaly, infantile cataracts.

Question 50

What is cretinism and what are its clinical features?

Answer 50

Cretinism = congential hypothyroidism - 6 P's: - Pot-bellied - Pale - Puffy-faced - Protruding umbilicus - Protuberant tongue - Poor brain development

Question 51

Which hormones are coupled to cAMP?

Answer 51

FLAT ChAMP - FSH - LH - ACTH - TSH - CRH - hCG - ADH - MSH - PTH

Question 52

Which hormones are coupled to cGMP?

Answer 52

BAD GraMPa (vasodilators) - BNP - ANP - EDRF (NO)

Question 53

Which hormones are coupled to IP3?

Answer 53

GOAT HAG - GnRH - Oxytocin - ADH (V1) - TRH - Histamine - ATII - Gastrin

Question 54

Which hormones are intracellular receptors?

Answer 54

(Steroid hormones): PET CAT on TV - Progesteron - Estrogen - Testosterone - Cortisol - Aldosterone - T3/T4 - Vitamin D

Question 55

Which hormones are coupled to a receptor tyrosine kinase?

Answer 55

Growth Factors - Insulin - IGF-1 - FGF - PDGF - EGF

Question 56

Which hormones are coupled to a nonreceptor tyrosine kinase?

Answer 56

PIGGLET - uses JAK/TAT pathway - Prolactin - Immunomodulators (e.g. IL-2, IL-6) - GH - G-CSF - Erythropoietin - Thrombopoietin

Question 57

Which cell-surface marker is associated with plasma cells?

Answer 57

CD27

Question 58

Which cell-surface marker is associated with B cells?

Answer 58

CD19, 20, 21, 40

Question 59

Which cell-surface markers are associated with NK cells?

Answer 59

CD56, CD16

Question 60

What is the CD4 cell surface marker associated with?

Answer 60

Regulatory and Helper T cells

Question 61

Which cell-surface markers are associated with regulatory T cells?

Answer 61

CD3, CD4, CD25 and FOXP3 (transcription factor)

Question 62

Ebstein Barr virus affects which cells?

Answer 62

Infects B cells (but peripheral smear features reactive/atypical cytotoxic T cells)

Question 63

Recipient’s cytotoxic T-lymphocytes destroy the graft within weeks to months of the transplant:

Answer 63

Acute graft rejection

Question 64

Vessel occlusion, ischemia, or fibrinoid necrosis, occurring within minutes to hours after a transplant:

Answer 64

Hyperacute graft rejection

Question 65

Donor peptides being displayed by recipient antigen-presenting cells, resulting in interstitial fibrosis, atherosclerosis, and vanishing bile duct syndrome:

Answer 65

Chronic organ rejection

Question 66

Which cell type is associated with CD34?

Answer 66

Pluripotent stem cell (Stimulated by G-CSF) --> G-CSF is often given to patients who have experienced myelosuppression as a result of chemotherapy.

Question 67

Which vasculitis is associated with asthma, sinusitis, skin nodules or purpura, peripheral neuropathy (eg, wrist/foot drop)?

Answer 67

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) - pANCA/MPO

Question 68

What is the classic pentad of thrombotic thrombocytopenic purpura (TTP) and what is it caused by?

Answer 68

TTP is a platelet disorder that is due to inhibition or deficiency of ADAMTS 13 (vWF metalloprotease) --> reduced degradation of vWF multimers (therefore more clotting) - Microangiopathic hemolytic anemia (MAHA) - Thrombocytopenia (because platelets are being used up to form the clot) - Neurologic abnormalities - Renal abnormalities - Fever

Question 69

What is the drug of choice for diagnosing myasthenia gravis?

Answer 69

Edrophonium (irreversible acetylcholinesterase inhibitor with a short duration of ~10-20 minutes)

Question 70

What is the classic triad of acute graft-versus-host disease (GVHD)?

Answer 70

Dermatitis, hepatitis (elevation in liver enzymes), and gastroenteritis

Question 71

Antimicrosomal autoantibodies

Answer 71

Hashimoto's Thyroiditis

Question 72

What is the triad (quad) of Plummer Vinson syndrome?

Answer 72

(IDEA) - Iron-deficiency anemia - Dysphagia - Esophageal webs - Atrophic glossitis

Question 73

What is the most common cause of death in infants born with congenital diaphragmatic hernia?

Answer 73

Pulmonary hypoplasia

Question 74

What treatment should be immediately initiated for heparin-induced thrombocytopenia (HIT)?

Answer 74

A direct thrombin inhibitor such as argatroban

Question 75

What is the mechanism of superantigens causing shock? (e.g. Staph aureus -TSST1 and Strep pyogenes-Exotoxin A)

Answer 75

Both involve binding to MHC II (remember exotoxins) and TCR outside of the antigen binding site, and cause an overwhelming release of IL-1, IL-2, IFN-gamma and TNF-alpha.

Question 76

Acute hemolytic transfusion reaction (anti-ABO antibodies) is what type of hypersensitivity reaction?

Answer 76

Type II

Question 77

Which cell types are involved in delayed-type hypersensitivity reactions?

Answer 77

CD4+ T lymphocytes and macrophages

Question 78

What is the mechanism by which eosinophils are involved in host defense during parasitic infection?

Answer 78

Antibody-dependent cell-mediated cytotoxicity, in which they recognize antibodies bound to the parasitic organism and release enzymes from their cytoplasmic granules.

Question 79

ALK mutation

Answer 79

Oncogene: | Receptor tyrosine kinase --> lung adenocarcinoma

Question 80

BCR-ABL mutation

Answer 80

Oncogene: | Tyrosine kinase --> CML, ALL

Question 81

BCL-2 mutation

Answer 81

Oncogene: | Anti-apoptotic molecule --> follicular and diffuse large B cell lymphoma

Question 82

APC

Answer 82

Tumor suppressor gene: | Negative regulator of Beta-catenin/WNT pathway --> Colorectal cancer (assoc. w/ FAP)

Question 83

BRCA1/BRCA2

Answer 83

Tumor suppressor: | DNA repair protein --> breast, ovarian and pancreatic cancer

Question 84

BRAF

Answer 84

oncogene; serine/threonine kinase; involved in melanoma and non-Hodgkin lymphoma

Question 85

c-kit

Answer 85

oncogene; cytokine receptor; involved in gastrointestinal stromal tumors (GIST); mutation indicates good candidacy for imatinib tx

Question 86

c-myc

Answer 86

oncogene; ch. 8; transcription factor; involved in Burkitt lymphoma [t(8;14)]

Question 87

HER2/neu

Answer 87

oncogene; tyrosine kinase; involved in breast, ovarian, and gastric carcinomas; mutation indicates good candidacy for trastuzumab

Question 88

L-myc

Answer 88

oncogene; transcription factor; involved in lung tumors (small cell)

Question 89

N-myc

Answer 89

oncogene; transcription factor; involved in neuroblastoma

Question 90

KRAS

Answer 90

oncogene; GTPase; colon, lung, and pancreatic cancer

Question 91

RET

Answer 91

oncogene; receptor tyrosine kinase; involved in MEN 2A (medullary thyroid cancer, pheochromocytoma, parathyroid hyperplasia) and 2B (medullary thyroid cancer, pheochromocytoma, mucosal neuromas), medullary thyroid cancer

Question 92

DCC

Answer 92

tumor suppressor gene; "deleted in colon cancer"

Question 93

DPC4/SMAD4

Answer 93

tumor suppressor gene; "deleted in pancreatic cancer"

Question 94

MEN1

Answer 94

tumor suppressor gene; ch. 11; menin; involved in MEN1 (parathyroid adenoma, pituitary adenoma, enteropancreatic tumors)

Question 95

NF1

Answer 95

ch. 17; tumor suppressor gene; neurofibronin, Ras GTPase activating protein; involved in neurofibromatosis type 1

Question 96

NF2

Answer 96

ch. 22; tummor suppressor gene: Merlin (schwannomin) protein; involved in neurofibromatosis type 2

Question 97

PTEN

Answer 97

tumor suppressor gene; Tyrosine phophatase of PIP3 (e.g. protein kinase B [AKT] activation); involved in breast cancer, prostate cancer, endometrial cancer

Question 98

Rb

Answer 98

tumor suppressor gene; inhibits E2F blocking G1-->S phase; involved retinoblastoma and osteosarcoma

Question 99

TP53

Answer 99

tumor suppressor gene; p53, activates p21, blocks G1-->S phase; involved in most human cancers, Li-Fraumeni Syndrome (a.k.a. SBLA cancer syndrome: sarcoma, breast, leukemia, adrenal gland)

Question 100

TSC1

Answer 100

tumor suppressor gene; hamartin protein; involved in tuberous sclerosis

Question 101

TSC2

Answer 101

tumor suppressor gene; tuberin(2berin) protein; involved in tuberous sclerosis

Question 102

VHL

Answer 102

tumor suppressor gene; inhibits hypoxia inducible factor 1a; involved in von Hippel-Lindau disease (chr. 3)

Question 103

WT1

Answer 103

tumor suppressor gene; transcription factor that regulates urogenital development (Wilms tumor - nephroblastoma)

Question 104

What are psammoma bodies and which neoplasms are associated with them?

Answer 104

Psammoma bodies are laminated, concentric spherules with dystrophic calcifications. The cancers in which they are seen can be remembered by the mnemonic "PSaMMoma": - Papillary carcinoma of the thyroid - Serous papillary cystadenocarcinoma of the ovary - Meningioma - Mesothelioma

Question 105

alkaline phosphatase

Answer 105

- metastases to bone or liver, Paget disease of bone, seminoma (placental ALP) - must exclude hepatic origin by checking LFTs and GGT levels

Question 106

CA-125

Answer 106

ovarian cancer

Question 107

CA 19-9

Answer 107

pancreatic adenocarcinoma

Question 108

CA 15-3/CA 27-29

Answer 108

breast cancer

Question 109

alpha-fetoprotein

Answer 109

- hepatocellular carcinoma - hepatoblastoma - yolk sac (endodermal sinus) tumor - mixed germ cell tumor - normally made by fetus, transiently elevated in pregnancy - high levels associated = neural tube and abdominal wall defects - low levels = down syndrome

Question 110

beta-hCG

Answer 110

- hydatidiform moles - choriocarcinomas (gestational trophoblastic disease) - testicular cancer - mixed germ cell tumor - produced by syncytiotrophoblasts of the placenta

Question 111

calcitonin

Answer 111

medullary thyroid carcinoma

Question 112

CEA

Answer 112

major: colorectal and pancreatic cancers minor: gastric, breast and medullary thyroid carcinomas

Question 113

chromogranin

Answer 113

neuroendocrine tumors

Question 114

PSA

Answer 114

prostate cancer

Question 115

S-100

Answer 115

melanoma (and other neural crest tumors including schwannomas)

Question 116

What is P-glycoprotein?

Answer 116

A transmembrane ATP-dependent efflux pump that is also known as multidrug resistance protein 1 (MDR1). Classically seen in adrenocortical carcinoma but can also be expressed by other cancer cells.

Question 117

What are common metastases to the brain?

Answer 117

Lung > Breast > Melanoma, Colon, Kidney

Question 118

What are common metastases to the liver?

Answer 118

Colon >> Stomach > Pancreas

Question 119

What are common metastases to bone?

Answer 119

Prostate, breast > Lung, Thyroid, Kidney

Question 120

Which cardiac anomaly results in a loud S1, a wide, fixed split S2?

Answer 120

Atrial septal defect (ASD)

Question 121

Which neoplasm commonly presents with DIC?

Answer 121

AML (especially M3 subtype)

Question 122

Spindle-shaped smooth muscle cells is most associated with which neoplasm?

Answer 122

Leiomyosarcoma

Question 123

Which neoplasm is characterized on histology as having large, halo-like cells, and what is there an increased risk for?

Answer 123

Paget disease of the nipple presents with characteristic large, halo-like cells on histologic exam, and carries a high risk of underlying in situ or invasive ductal carcinoma.

Question 124

What is TRAP and what is it associated with?

Answer 124

TRAP = tartrate-resistant acid phosphatase and is associated with Hairy Cell Leukemia

Question 125

What is the mode of inheritance of glucose-6-phosphate dehydrogenase deficiency?

Answer 125

X-linked recessive

Question 126

What causes a porcelain gallbladder, and what does this manifestation have an increased risk for?

Answer 126

Chronic cholecystitis; increased risk for gallbladder adenocarcinoma

Question 127

What is the equation for the half-life of a drug eliminated via first-order pharmacokinetics?

Answer 127

t1/2 = (0.693 x Vd)/CL

Question 128

What is the equation used to calculated oral bioavailability?

Answer 128

F = AUC (oral) / AUC (IV)

Question 129

What is the equation for loading dose?

Answer 129

Loading dose = (Cp x Vd) / F - Cp = target plasma concentration - Vd = volume of distribution - F = oral bioavailability

Question 130

What is the equation for maintainence dose?

Answer 130

Maintenance dose = (Cp x CL X τ) / F - Cp = target plasma concentration - CL = clearance - τ = dosage interval (time between doses) - F = oral bioavailability

Question 131

Which anti-arrhythmics can lead to torsades de pointes?

Answer 131

"Type 1A and III prolong QT" - e.g. Type IA Quinidine, Procainamide, Disopyramide - e.g. Type III Ibutilide

Question 132

What is bethanechol and what is it commonly used for?

Answer 132

Bethanechol is a cholinomimetic that stimulates muscarinic receptors, but has no effect on nicotinic receptors. It is commonly used for postoperative ileus and neurogenic bladder.

Question 133

What are all the clinical uses of somatostatin (octreotide)?

Answer 133

Acromegaly, carcinoid syndrome, gastrinoma, glucagonoma, esophageal varices.

Question 134

What is the MOA of fibrates (e.g. gemfibrozil)?

Answer 134

Gemfibrozil is a fibrate that stimulates synthesis of lipoprotein lipase by activation of the PPAR-a protein. In turn, lipoprotein lipase increases hydrolysis of triglycerides and VLDL, which ultimately results in decreased triglyceride and LDL levels

Question 135

What is the MOA of nitroglycerin?

Answer 135

Vasodilate by NO in vascular smooth muscle in cGMP and smooth muscle relaxation. Dilate veins >> arteries --> decrease preload (and therefore decrease myocardial oxygen demand).

Question 136

What is the mechanism of toxicity of acetaminophen overdose?

Answer 136

Acetaminophen metabolite (NAPQI) depletes glutathione and forms toxic tissue byproducts in liver. N-acetylcysteine is antidote—regenerates glutathione.

Question 137

What is the MOR of aminoglycosides?

Answer 137

The mechanism for aminoglycoside resistance is covalent modification via acetylation, adenylation, or phosphorylation.

Question 138

What are the most common chelators used for lead poisoning?

Answer 138

Dimercaprol, EDTA, or succimer

Question 139

Which chelator is commonly used for copper toxicity?

Answer 139

Penicillamine (can also be used for lead poisoning)

Question 140

Which SERM carries an increased risk of endometrial cancer, and which one does not?

Answer 140

Tamoxifen increases risk of endometrial cancer. Raloxifene does not, so you can "relax"

Question 141

What are the mechanisms of action of tiotropium and ipratropium bromide?

Answer 141

They are M3 muscarinic antagonists.

Question 142

Which HIV drugs can cause gastrointestinal intolerance and lipodystrophy?

Answer 142

Protease inhibitors (e.g. lopinavir-ritonavir, amprenavir, nelfinavir, indinavir, and saquinavir).

Question 143

Which drugs increase the risk of digoxin toxicity?

Answer 143

Drugs that displace digoxin from tissue-binding sites, and clearance (eg, verapamil, amiodarone, quinidine)

Question 144

What are signs of a salicylate overdose?

Answer 144

Salicylate (aspirin) overdose causes tinnitus; a combined metabolic acidosis (late) and respiratory alkalosis (early) will also develop.

Question 145

What is the most common manifestation of amiodarone toxicity?

Answer 145

Hypothyroidism (Pulmonary fibrosis, hepatotoxicity, and bluish skin discoloration are other less common, classic manifestations of amiodarone toxicity)

Question 146

What is the mechanism of action of entacapone?

Answer 146

Entacapone is a COMT inhibitor that increases the bioavailability of levodopa by inhibiting its peripheral methylation.

Question 147

Adding allopurinol to the treatment regimen of someone being treated with antimetabolites, will do what to the concentration of which metabolites?

Answer 147

Adding allopurinol to the regimen of a patient being treated with azathioprine and 6-mercaptopurine, will cause an increase in their concentration (because allopurinol inhibits xanthine oxidase, which prevents their metabolism).

Question 148

What is the antidote for serotonin syndrome?

Answer 148

Cyproheptadine

Question 149

Which antihypertensive agent(s) can most successfully prevent progression to diabetic nephropathy?

Answer 149

ACE inhibitors and ARBs

Question 150

What is the MOA of digoxin in initiating ventricular rate control?

Answer 150

Digoxin is used for ventricular rate control in atrial fibrillation as it decreases atrioventricular nodal conduction by increasing parasympathetic vagal tone.

Question 151

What is the most common adverse side effect of thrombolytic use (e.g. alteplase)

Answer 151

Hemorrhage

Question 152

What is the most serious complication/ADR of metformin use?

Answer 152

Lactic acidosis (therefore contraindicated in patients with renal insufficiency)

Question 153

What is the most common cause of bloody nipple discharge and how is it described histologically?

Answer 153

Intraductal papilloma is the most common cause of bloody nipple discharge; it is often described as a proliferation of papillary cells in a cystic wall or duct, that may contain focal atypia.

Question 154

What is the initial drug of choice for status epilepticus?

Answer 154

Benzodiazepines (e.g. lorazepam)

Question 155

What is the preferred treatment for restless leg syndrome?

Answer 155

Dopamine agonists (e.g. ropinirole, pramipexole)

Question 156

What are symptoms of digoxin toxicity?

Answer 156

Most common: life-threatening arrhythmia GI: anorexia, nausea and vomiting, abdominal pain Neurologic: fatigue, confusion, weakness, color vision alterations

Question 157

What is the mechanism by which niacin causes cutaneous flushing? What can b given to patients to reduce this SE?

Answer 157

- Niacin increases prostaglandin release | - Give NSAIDs

Question 158

Which class of lipid-lowering agents causes an increase in TG synthesis?

Answer 158

Bile acid resins (e.g. chlestyramine, colestipol, colesevelam)

Question 159

Long term survival in patients following heart failure due to left ventricular dysfunction, is most improved with use of:

Answer 159

beta blockers

Question 160

What effect do corticosteroids have on neutrophils, eosinophils and lymphocytes?

Answer 160

Corticosteroids cause neutrophilia, eosinopenia and lymphopenia. Corticosteroids cause neutrophilia by decreasing the activation of neutrophil adhesion molecules, therefore impairing migration out of the vasculature into sits of inflammation. The eosinopenia and lymphopenia are caused by sequestration in lymph noeds and apoptosis, respectively.

Question 161

What are some of the applications and ADRs of clonidine?

Answer 161

Clonidine is an alpha-2 agonist that decreases sympathetic outflow from the central nervous system. Uses: hypertensive urgency (when refractory to other agents) ADRs: CNS depression, bradycardia, hypotension, respiratory depression, miosis, rebound hypertension (with abrupt cessation)

Question 162

What are some of the applications and ADRs of alpha-methyldopa?

Answer 162

The only actual implication for alpha-methyldopa is for hypertension in pregnancy. ADRs: Direct Coombs (+) hemolysis - autoimmune hemolytic anemia; SLE-like syndrome (drug-induced lupus confirmed with positive anti-histone).

Question 163

Which drugs are inducers of CYP450?

Answer 163

CHRONIC ALCOHOLics STeal PHEN-PHEN and NEver Refuse GReasy CARBS: - Chronic alcohol use - St. John's wort - Phenytoin - Phenobarbital - Nevirapine - Rifampin - Griseofulvin - Carbamazepine

Question 164

Which drugs are inhibitors of CYP450?

Answer 164

SICKFACES.COM - Sodium valproate - INH - Cimetidine - Ketoconazole - Fluconazole - Acute alcohol abuse - Chloramphenicol - Erythromycin (macrolides) - Sulfonamides - Ciprofloxacin - Omeprazole - Metronidazole

Question 165

Pt with depression takes an SSRI. What is the typical suffix for this class of drug?

Answer 165

-etine (eg, fluoxetine)

Question 166

A man with hyperlipidemia takes an HMG-CoA reductase inhibitor. What is the typical suffix for this class of drug?

Answer 166

-statin (eg, atorvastatin)

Question 167

A pt takes an antibiotic that functions as a transpeptidase inhibitor. What is the typical suffix for this class of drug?

Answer 167

-cillin (eg, ampicillin); binds penicillin-binding protein

Question 168

A pt takes a drug for erectile dysfunction that inhibits PDE-5. What is the typical suffix for this class of drug?

Answer 168

-afil (eg, sildenafil)

Question 169

A pt receives an inhaled general anesthetic. What is the typical suffix for this class of drug?

Answer 169

-ane (eg, halothane)

Question 170

A pt takes a benzodiazepine. What are the 2 typical suffixes for this class of drug?

Answer 170

-azepam (eg, diazepam) or -zolam (eg, alprazolam)

Question 171

A pt takes a drug for a fungal infection that inhibits ergosterol synthesis. What is the typical suffix for this class of drug?

Answer 171

-azole (eg, ketoconazole)

Question 172

A pt takes an antibiotic that inhibits protein synthesis and discolors teeth. What is the typical suffix for this class of drug?

Answer 172

-cycline (eg, tetracycline)

Question 173

A pt receives a local anesthetic. What is the typical suffix for this class of drug?

Answer 173

-caine (eg, lidocaine)

Question 174

A pt w/HIV takes a protease inhibitor. What is the typical suffix for this class of drug?

Answer 174

-navir (eg, ritonavir)

Question 175

A pt w/CHF takes a β-blocker. What is the typical suffix for this class of drug?

Answer 175

-olol (eg, propranolol)

Question 176

A pt w/schizophrenia takes a typical antipsychotic. What is the typical suffix for this class of drug?

Answer 176

-azine (eg, thioridazine)

Question 177

A pt receives a barbiturate for sedation during a procedure. What is the typical suffix for this class of drug?

Answer 177

-barbital (eg, phenobarbital)

Question 178

A pt w/Tx-resistant depression takes tricyclic antidepressants. What is the typical suffix for this class of drug?

Answer 178

-triptyline (eg, amitriptyline) or -ipramine (eg, imipramine)

Question 179

A pt w/DM takes an angiotensin-converting enzyme (ACE) inhibitor. What is the typical suffix for this class of drug?

Answer 179

-pril (eg, captopril)

Question 180

A pt w/asthma takes a β2-agonist. What is the typical suffix for this class of drug?

Answer 180

-terol (eg, albuterol)

Question 181

A pt w/GERD takes an H2-antagonist. What is the typical suffix for this class of drug?

Answer 181

-tidine (eg, cimetidine)

Question 182

A pt w/growth hormone deficiency takes a pituitary hormone as therapy. What is the typical suffix for pituitary hormones?

Answer 182

-tropin, (eg, somatotropin)

Question 183

A pt w/prostatic hyperplasia takes an α1-antagonist. What is the typical suffix for this class of drug?

Answer 183

-zosin (eg, prazosin)

Question 184

A pt w/DM takes a PPAR-γ activator. What is the typical suffix for this class of drug?

Answer 184

-glitazone (eg, rosiglitazone)

Question 185

A pt w/osteoporosis is prescribed a bisphosphonate. What is the typical suffix for this class of drug?

Answer 185

-dronate (eg, alendronate)

Question 186

A pt w/GERD takes a proton pump inhibitor. What is the typical suffix for this class of drug?

Answer 186

-prazole (eg, omeprazole)

Question 187

A pt w/glaucoma takes a prostaglandin analog. What is the typical suffix for this class of drug?

Answer 187

-prost (eg, latanoprost)

Question 188

A pt w/HTN takes an angiotensin-II receptor blocker. What is the typical suffix for this class of drug?

Answer 188

-sartan (eg, losartan)

Question 189

A pt w/urinary retention after surgery takes a cholinergic agonist. What is the typical suffix for this class of drug?

Answer 189

-chol (eg, bethanechol, carbachol)

Question 190

A pt undergoing surgery receives a nondepolarizing paralytic. What are 2 typical suffixes for this class of drug?

Answer 190

-curium (eg, atracurium) or -curonium (eg, vecuronium)

Question 191

A pt w/myasthenia gravis takes an AChE inhibitor. What is the typical suffix for this class of drug?

Answer 191

-stigmine (eg, neostigmine)

Question 192

A pt w/migraines takes a 5-HT1B/1D agonist. What is the typical suffix for this class of drug?

Answer 192

-triptan (eg, sumatriptan)

Question 193

A pt w/a herpes infection takes an antiviral DNA polymerase inhibitor. What is the typical suffix for this class of drug?

Answer 193

-ovir (eg, acyclovir)

Question 194

A pt w/HTN starts taking a dihydropyridine Ca2+ channel blocker. What is the typical suffix for this class of drug?

Answer 194

-dipine (eg, amlodipine)

Question 195

A pt w/atypical pneumonia takes a macrolide antibiotic. What is the typical suffix for this class of drug?

Answer 195

-thromycin (eg, azithromycin)

Question 196

A pt w/the flu receives a neuraminidase inhibitor. What is the typical suffix for this class of drug?

Answer 196

-ivir (eg, oseltamivir)

Question 197

A pt w/pinworms receives an antiparasitic/antihelminthic agent. What is the typical suffix for this class of drug?

Answer 197

-bendazole (eg, mebendazole)

Question 198

A pt w/atrial fibrillation starts taking a direct factor Xa inhibitor. What is the typical suffix for this class of drug?

Answer 198

-xaban (eg, apixaban, edoxaban, rivaroxaban)

Question 199

A pt w/a recent kidney transplant takes a chimeric (human-mouse) monoclonal Ab. What is the typical suffix for this class of drug?

Answer 199

-ximab (eg, rituximab); suffix for chimeric human-mouse monoclonal Ab

Question 200

A pt w/a recent kidney transplant takes a humanized mouse monoclonal Ab. What is the typical suffix for this class of drug?

Answer 200

-zumab (eg, bevacizumab); suffix for humanized mouse monoclonal Ab

Question 201

A pt w/chronic myelogenous leukemia starts taking a tyrosine kinase inhibitor. What is the typical suffix for this class of drug?

Answer 201

-tinib (eg, imatinib)

Question 202

How do the alkylating agents cyclophosphamide and ifosfamide cause hemorrhagic cystitis? What is it treated with and what is the MOA of that drug?

Answer 202

The condition is a result of the renal excretion of acrolein, a hepatic metabolite of these drugs. Treat with mesna - a sulfhydryl compound that is oxidized to an inactive form but is reactivated in the kidney. Once in the urine, it binds to acrolein to form an inert compound that is excreted, preventing toxic exposure to the bladder epithelium.

Question 203

At what point after ischemia to the myocardial tissue does myocyte injury become irreversible?

Answer 203

Myocyte injury becomes irreversible after about 20 minutes of oxygen starvation.

Question 204

What is the MOA of cromolyn?

Answer 204

"Lynn's Bee Control" | - Prevents mast cell degranulation, inhibiting the release of histamine.

Question 205

What is the MOA of macrolides?

Answer 205

Macrolides act on the bacterial 50s ribosomal subunit and block translocation (specifically by binding to the 23S rRNA)

Question 206

Which lab and exam findings might suggest multiple myeloma?

Answer 206

CRAB - HyerCalemia - Renal involvement - Anemia - Bone lytic lesions/back pain

Question 207

What is MGUS?

Answer 207

Monoclonal Gammopathy of Undetermined Significance: - An idiopathic, monoclonal expansion of plasma cells (M spike); asymptomatic but can lead to multiple myeloma in 1-2% of patients each year - No CRAB findings

Question 208

What is the first-line treatment for antipsychotic-induced acute dystonia and for most other EPSs associated with antipsychotic medications?

Answer 208

Anticholinergic agents, such as benztropines, are the first-line treatment. Anticholinergics are often given prophylactically with the initial administration of antipsychotics to prevent the onset of acute dystonia.

Question 209

What are some signs and symptoms of hypoglycemia?

Answer 209

* Neurogenic Symptoms: - Adrenergic (catechol mediated): tremor, palpitations, anxiety - Cholinergic (ACh-mediated): cold sweats, hunger, paresthesias * Neuroglycopenic Symptoms (due to low glucose in the brain): - General weakness, confusion, drowsiness, syncope, difficulty speaking, blurry vision

Question 210

What is Liddle Syndrome?

Answer 210

Liddle syndrome is a genetic condition characterized by a GOF mutation in the epithelial ENaC on the collecting tubule, leading to early hypertension characterized by low levels of renin and angiotensin due to negative feedback. Reabsorption of sodium without an accompanying anion creates a negative electrical gradient across the luminal membrane, causing increased secretion of potassium and hydrogen ions; this results in hypokalemia and metabolic alkalosis (note the patient’s elevated pH and bicarbonate levels). Liddle syndrome is commonly treated with a diuretic that inhibits ENaC, such as triamterene or amiloride.

Question 211

Which major complication can arise from use of oral or IV corticosteroids, such as methylprednisolone, a common treatment for ulcerative colitis flares?

Answer 211

Avascular necrosis (esp. of femoral head). Other complications include: abdominal striae and increased girth, suppression of the hypothalamic-pituitary-adrenal axis, hyperglycemia, osteoporosis, moon facies, buffalo hump, immunosuppression, and impaired wound healing

Question 212

What is the gold standard chemotherapy regimen for treating Hodgkin lymphoma?

Answer 212

The AVBD protocol: - Adriamycin (doxorubicin) - Bleomycin - Vinblastine - Dacarbazine

Question 213

In which form of IBD are non-caseating granulomas seen?

Answer 213

Chron Disease

Question 214

Graves' disease is an example of which type of hypersensitivity?

Answer 214

Type II

Question 215

Goodpasture Syndrome is an example of which type of hypersensitivity?

Answer 215

Type II: anti-glomerular basement membrane antibodies (anti-GBM Ab), are autoantibodies against the alpha 3 chain of type IV collagen found in the glomerular basement membrane and pulmonary capillary membrane.

Question 216

Which portal ↔ systemic shunt is responsible for esophageal varices?

Answer 216

Left gastric ↔ azygos vein

Question 217

Which portal ↔ systemic shunt is responsible for caput medusae varices?

Answer 217

Paraumbilical ↔ small epigastric veins of the anterior abdominal wall

Question 218

Which portal ↔ systemic shunt is responsible for anorectal varices?

Answer 218

Superior rectal ↔ middle and inferior rectal

Question 219

A Pancoast Tumor can cause what spinal cord lesion, and consequently which syndrome?

Answer 219

Pancoast tumor can impinge on the stellate ganglion causing Horner Syndrome (ptosis, miosis, anhidrosis).

Question 220

What is the pathophysiology of proteinuria seen in minimal change disease?

Answer 220

Disruption of the filtration barrier which is composed of heparan sulfate (negatively charged molecule which repels other (-) charges [e.g. albumin]).

Question 221

What happens to TBG (thyroid-binding globulin) in states of high estrogen (e.g. pregnancy, OCP use)?

Answer 221

TBG increases, which causes total T4 to increase. FREE T4 remains unchanged (due to normal negative feedback).

Question 222

What is the difference between Langerhans cells and Langhans "giant" cells?

Answer 222

Langhans giant cells are found in granulomatous lung disease, whereas Langerhans cells are found specifically in pulmonary Langerhans cell histiocytosis.

Question 223

How does succinylcholine differ from pancuronium, tubocurarine, and vecuronium?

Answer 223

Succinylcholine is a depolarizing NMJ blocker; therefore with the TOF test (train-of-four) it first exhibits an equal (lower across the board) attenuation of the motor endplate's response to stimulation, followed by a faded phase II response. Pancuronium, tubocurarine and vecuronium are all non-depolarizing, which means that they block pre-synaptic ACh release, thus resulting in an immediate faded TOF response.

Question 224

What is the first-line treatment for an acute attack of gouty arthritis?

Answer 224

NSAIDs

Question 225

Which of the lipid-lowering agents can precipitate gouty arthritis?

Answer 225

Niacin - via increased renal excretion of uric acid

Question 226

What is octreotide and what is it used for?

Answer 226

Octreotide is a somatostatin analog, and is often used to treat the symptoms of carcinoid syndrome (e.g. secretory diarrhea).

Question 227

What are symptoms of alcohol withdrawal and how is it treated?

Answer 227

Alcohol withdrawal symptoms: - 3–36 hours: minor symptoms similar to other depressants: anxiety, tremor "the shakes", sweating - 6–48 hours: withdrawal seizures - 12–48 hours: alcoholic hallucinosis - 48–96 hours: delirium trmns Treatment: benzodiazepines! (esp. long-acting due to self-tapered withdrawal effect: chlordiazepoxide, lorazepam, diazepam)

Question 228

What is the mechanism by which nitrates causes vasodilation?

Answer 228

Nitrates (e.g. nitroglycerin, isosorbide dinitrate, isosorbide mononitrate) activate guanylyl cyclase which subsequently increases cGMP and causes dephosphorylation of myosin light-chain kinase (causes smooth muscle relaxation).

Question 229

Which of the antibiotics has weak MAO inhibitor activity? What is a potential ADR.

Answer 229

Linezolid Can cause serotonin syndrome.

Question 230

What are the 4 "P's" that can be used to treat thyroid storm?

Answer 230

- Propranolol (for adrenergic effects and prevents peripheral conversion of T4 to T3) - Propylthiouracil (also blocks T4 to T3) - Prednisolone - Potassium iodide

Question 231

What is the MOA of oseltamivir?

Answer 231

Oseltamivir (Tamiflu) is a neuraminidase inhibitor. It can be used to treat or prevent the flu by inhibiting the release of newly formed virions.

Question 232

Why are tricyclic anti-depressants contraindicated in the elderly?

Answer 232

TCADs have strong anticholinergic effects (e.g. confusion, urinary retention, constipation), which can exacerbate those caused by comorbid conditions (e.g. BPH, dementia).

Question 233

Which anesthetics exhibit hepatotoxicity?

Answer 233

Halogenated inhaled anesthetics (e.g. halothane, enflurane).

Question 234

Which infection are HIV (and immunocompromised) patients at increased risk for if their CD4+ count is < 50? What is the best prophylactic treatment?

Answer 234

Mycobacterium avium complex. Prophylaxis: macrolide (e.g. azithromycin)

Question 235

Which lipid-lowering agent class is the most protective of cardiovascular events?

Answer 235

Statins (HMG-CoA reductase inhibitors)at is

Question 236

What is the best measure/estimator of GFR?

Answer 236

Inulin clearance. Inulin is only filtered at the glomerulus, it is neither secreted nor reabsorbed. Creatinine is often used clinically because it is easier to measure; however, it slightly overestimates GFR because it is also secreted. (CREatinine is seCREted).

Question 237

What is the equation for RBF?

Answer 237

RBF = renal blood flow --> it is the amount of blood (i.e. RBCs only) that is flowing through the kidney. If we know renal plasma flow (includes RBCs and ECF plasma) we can calculate RBF. RBCs = Hct, therefore plasma = 1-Hct. RBF = RPF/(1-Hct)

Question 238

What is filtration fraction and what is a normal value?

Answer 238

FF = GFR/RPF Normal: 20%

Question 239

At what plasma concentration of glucose can one begin to observe glucosuria in an adult?

Answer 239

200 mg/dL

Question 240

The PCT of the nephron exhibits nearly 100% reabsorption of which two substances?

Answer 240

Glucose and Amino Acids

Question 241

Where and how does PTH affect solute reabsorption on the nephron?

Answer 241

- PO4^3-: PTH inhibits Na+/PO4^3- cotransport in the PCT | - Ca2+: PTH increases Ca2+/Na+ exchanger activity on the basolateral side of the DCT

Question 242

What do RBC casts indicate?

Answer 242

Glomerulonephritis, malignant HTN

Question 243

What do WBC casts indicate?

Answer 243

Tubulointerstitial inflammation, acute pyelonephritis, transplant rejection.

Question 244

What do fatty casts indicate?

Answer 244

Nephrotic syndrom

Question 245

What do granular ("muddy brown") casts indicate?

Answer 245

Acute tubular necrosis

Question 246

What do waxy casts indicate?

Answer 246

End-stage renal disease/Chronic renal failure

Question 247

What do hyaline casts indicate?

Answer 247

Nonspecific; can be a normal finding and is often seen in concentrated urine samples

Question 248

What is the most common cause of primary nephrotic syndrome in Caucasian adults?

Answer 248

Membranous nephrophathy

Question 249

What do Kimmelstiel-Wilson bodies classically indicate?

Answer 249

Diabetic glomerulonephropathy

Question 250

What is the WAGR complex?

Answer 250

Related to Wilms tumor/chr 11: - Wilms tumor - Aniridia (absence of iris) - Genitourinary malformations - Retardation/intellectual disability

Question 251

What may painless hematuria indicate?

Answer 251

Transitional cell carcinoma (bladder cancer) - There are no casts because the cancer occurs after the kidneys - Associated with Pee SAC: - Phenacetin - Smoking - Aniline dyes - Cyclophosphamide (hemorrhagic cystitis)

Question 252

What does an increased BUN/Cr ratio indicate?

Answer 252

Pre-renal azotemia - Hypotension/dehydration lowers the RBF, which lowers GFR. In an attempt to conserve water, H2O reabsorption (and subsequently BUN) is increased. (Also results in an decreased FENa since the kidney reabsorbs more Na along with water and other solutes)

Question 253

What does a decreased BUN/Cr ratio indicate?

Answer 253

Intrinsic renal failure - An intrinsic issue with the kidney (e.g. tubular necrosis, ischemia/toxins) impairs the kidneys ability to reabsorb BUN. (Also results in an increased FENa since the kidney is unable to reabsorb Na)

Question 254

What is acute interstitial nephritis (tubulointerstitial nephritis)?

Answer 254

A drug induced nephritis characterized by interstitial renal inflammation. Pyuria is seen with eosinophils, due to the fact of this being a Type I hypersensitivity reaction. Common culprits include: NSAIDs, diuretics, penicillins, PPIs, rifampin.

Question 255

How does ADPKD lead to berry aneurysms?

Answer 255

ADPKD is characterized by polycystic kidneys which secrete excess renin and subsequently cause hypertension. This HTN can increase development/risk of aneurysms. Most causes of death from ADPKD are related to HTN.

Question 256

Which vitamin can cause pseudotumor cerebri?

Answer 256

Vitamin A Vitamin A is a precursor to CSF production. Therefore, in excess, it can produces pseudotumor cerebri.

Question 257

Which class of diuretics is sulfa based? Which is the exception that can be used in patients with a sulfa allergy?

Answer 257

Loop diuretics e.g. furosemide, bumetanide, and torsemid are all sulfa basd. Ethacrynic acid, is a nonsulfonamide inhibitor.

Question 258

Goodpasture Syndrome is an example of which type of nephritic syndrome?

Answer 258

Rapidly progressive (crescentic) glomerulonephritis.

Question 259

What drug should be given with chemotherapy to prevent tumor lysis syndrome?

Answer 259

Allopurinol - it prevents the conversion of purines to uric acid.

Question 260

What is first-line treatment for an uncomplicated UTI in a patient with a sulfa allergy?

Answer 260

Nitrofurantoin (If not allergy, use TMP-SMX)

Question 261

Episodic gross hematuria that is concurrent with a respiratory or GI infection is likely indicative of?

Answer 261

IgA nephropathy (Berger disease) - the most common cause of nephropathy worldwide

Question 262

What is Conn syndrome and what is the best treatment?

Answer 262

Conn Syndrome is primary hyperaldosteronism. Treated with an aldosterone antagonist (e.g. spironolactone).

Question 263

Where in/from the kidney is renin secreted?

Answer 263

Juxtaglomerular cells in the tunica media of the afferent arteriole.

Question 264

Which immunosuppressant is classically nephrotoxic?

Answer 264

Cyclosporin - MOA: inhibits calcineurin by binding/enhancing cyclophilin. By preventing IL-2 transcription, T-cell activation is effectively blocked. Nephotoxicity likely occurs due to impairment of endothelial cell function, leading to reduced production of vasodilators (prostaglandins and nitric oxide) and enhanced release of vasoconstrictors (endothelin and thromboxane) --> reduces RBF.

Question 265

What happens to the afferent and the efferent arterioles in states of severe volume depletion?

Answer 265

Both the afferent and efferent arterioles constrict --> sympathetic response to preserve blood pressure.

Question 266

What are the "4" symptoms of hypercalcemia?

Answer 266

"Stones, bones, groans and psychiatric overtones" - Stones = kidney stones - Bones = bone pain due to increased turnover - Groans = abdominal pain - Psychiatric overtones = anxiety, altered mental status

Question 267

What are the equations for CO (cardiac output)?

Answer 267

CO = SV x HR CO = (rate of oxygen consumption) / (arterial oxygen - venous oxygen)

Question 268

What is the equation for stroke volume?

Answer 268

SV = EDV - ESV SV = CO/HR (HR can be estimated by the RR ratio, which is 60/RR in sec/beat)

Question 269

How does Cushing disease differ from Cushing syndrome?

Answer 269

Cushing disease refers specifically to a ACTH-secreting pituitary adenoma. Cushing syndrome refers to the general symptoms of cortisol excess (HTN, weight gain, moon facies, abdominal striae, truncal obesity, buffalo hump, etc.)

Question 270

How can you distinguish Wilms tumor from a Neuroblastoma on palpation?

Answer 270

WilmS tumor is Smooth and unilateral NeuroBlastoma is Bumpy/irregular and can cross the midline

Question 271

What happens if you removal the adrenal glands of a patient who has a ACTH-secreting pituitary adenoma?

Answer 271

This is the pathophysiology of Nelson Syndrome Removal of the adrenal glands in someone who has Cushing's Disease, removes the negative feedback on the pituitary, causing pituitary hypertrophy and increased mass effect (headache, hyperpigmentation, bitemporal hemianopia).

Question 272

Patient is presenting with euvolemic hyponatremia. What is the the most likely diagnosis and what is the pathophysiology of this condition?

Answer 272

SIADH (Syndrome of inappropriate antidiuretic hormone secretion) - Excessive free water retention, with continued Na+ excretion - The body responds to water retention with a decrease in aldosterone and increase in ANP and BNP --> kidneys will secrete Na+ since water will follow, which corrects the volume status; however, this doesn't correct Na+ lvels - Hyponatremia must be corrected slowly to prevent osmotic demyelination syndrome - Treatmnt: fluid restriction, salt tablets, conivaptan (VAsoPression ANtangonists)

Question 273

What are the 4 ways to diagnose diabetes mellitus?

Answer 273

- Hemoblogin A1C ≥ 6.5% - Fasting glucose (8-hour) ≥ 126 mg/dL - 2-hour Oral Glucose Tolerance Test (OGTT) ≥ 200 mg/dL - Random glucose ≥ 200mg/dL with symptoms

Question 274

Describe the type and presentation of the neuroendocrine tumor associated with pancreatic alpha cells?

Answer 274

Glucagonoma Presents like diabetes. 4 D's: - Diabetes (hyperglycemia due to excess glucagon) - Dermatitis (necrolytic migratory erythema) - Declining weight - DVT

Question 275

Describe the type and presentation of the neuroendocrine tumor associated with pancreatic beta cells?

Answer 275

Insulinoma Presents with hypoglycemia. Symptomatic patients will have low blood glucose and increased C-peptide (vs. exogenous insulin). 10% associated with MEN1.

Question 276

Describe the type and presentation of the neuroendocrine tumor associated with pancreatic delta cells?

Answer 276

Somatostatinoma Presents with an overproduction of somatostatin which basically halts the entire digestive system (i.e. prevents/downregulates secretion of secretin, insulin, glucagon, CCK, gastrin and GIP). This can lead to steatorrhea and gallstones (from decreased CCK), and achlorhydria (low H+ production from decreased secretin).

Question 277

Which Vitamin deficiency may be present with Carcinoid syndrome?

Answer 277

Vitamin B3 - can cause pellagra Remember, carcinoid syndrome results in excess 5-HT production. Since Tryptophan is a precursor to both serotonin and niacin, carcinoid syndrome shunts all of the Tryptophan into making serotonin, causing a relative deficiency of B3.

Question 278

Which tumors are associated with MEN1?

Answer 278

MEN1 = menin gene (Chr 11 tumor suppressor) 3 P's: - Pituitary adenoma - Pancreatic endocrine tumors (e.g. ZE, insulinomas, VIPomas) - Parathyroid adenomas

Question 279

Which tumors are associated with MEN2A?

Answer 279

``` RET mutation - receptor tyrosine kinase: 2 P's: - Pheochromocytoma - Pituitary adenoma - Medullary thyroid carcinoma ```

Question 280

Which tumors are associated with MEN2B

Answer 280

``` RT mutation; associated with Marfinoid habitus 1 P: - Pheochromocytoma - Medullary thyroid carcinoma - Mucosal neuromas ```

Question 281

Which diabetes drug produces modest weight loss and is often considered the safest? What is the MOA of this drug?

Answer 281

Insulin MOA: prevents gluconeogenesis, increases glycolysis and increases insulin sensitivity via peripheral glucose uptake. ADR: can cause GI and lactic acidosis (due to decreased conversion of lactate to glucose in the liver via the cori cycle, which leads to a build up of lactate in muscle).

Question 282

Which drug class does the drug tolbutamide belong to and what is this class' MOA?

Answer 282

Tobutamide and other "ides" = sulfonylurea MOA: increase endogenous insulin release by close the K+ channel in the beta cell membrane (depolarization leads to increase insulin release via Ca2+ influx)

Question 283

What drug class does pioglitazone belong to and what is it's MOA?

Answer 283

"Glitazones" aka thiazolidinediones MOA: increase insulin sensitivity in peripheral tissue via upregulation of PPAR-γ (perioxisome proliferated-activated R gamma) ADR: weight gain (via decreased leptin), edema, HF

Question 284

How do the sulfonylureas and the meglitinides differ?

Answer 284

They have the same MOA (bind and inhibit the K+ channel) but bind at different sites.

Question 285

What drug class does exenatide belong to and what is it's MOA?

Answer 285

GLP-1 analog MOA: increase glucose-dependent insulin release

Question 286

What drug class does saxagliptin belong to and what is it's MOA?

Answer 286

Saxagliptin = Onglyza a DPP-4 inhibitor MOA: DPP-4 is an enzyme that normally deactivates GLP-1; therefore by inhibiting DPP-4, these drugs increase glucose-dependent insulin release. They are weight neutral!

Question 287

What drug class does pramlintide belong to and what is it's MOA?

Answer 287

PrAMLINtide is an AMYLIN analog. MOA: Decrease gastric empyting and glucacong release

Question 288

What drug class does canagliflozin belong to and what is it's MOA?

Answer 288

Sodium-glucose co-transporter 2 (SGLT2) inhibitor MOA: block reabsorption of glucose in PCT ("lozin" glucose in the urine --> can cause UTI/yeast infections)

Question 289

What drug class does acarbose belong to and what is it's MOA?

Answer 289

α-glucosidase inhibitor MOA: inhibit intestinal brush-border α-glucosidase leading to decreased intestinal absorption (decrease post-prandial hypoglycemia)

Question 290

What are the two most commonly thioamides are how do they work? How do they differ?

Answer 290

PTU (propylthiouracil) and methimazole are both used to treat hyperparathyroidism by blocking thyroid peroxidase. They work by inhibiting the oxidation of iodide and organification/coupling of iodine. PTU also blocks 5'-deiodinase and is safe to use in Pregnancy. Methimazole is teratogenic. ADRs: agranulocytosis, aplastic anemia, hepatotoxicity

Question 291

What are the most common manifestations of prolactinoma or hyperprolactinemia in female vs. male patients?

Answer 291

Males: present with diminished libido Females: present with amenorrhea

Question 292

Hypercalcemia is a known paraneoplastic syndrome, most commonly associated with which cancer?

Answer 292

Squamous cell carcinoma of the lung

Question 293

Which cartilage, muscles and nerves does the 1st pharyngeal arch give rise to?

Answer 293

All of the "M's" Cartilage: - Maxillary process → Maxilla, zygoMatic bone - Mandibular process → Meckel cartilage → Mandible, Malleus and incus, sphenoMandibular ligament Muscles: - Muscles of Mastication (temporalis, Masseter, lateral and Medial pterygoids), Mylohyoid, anterior belly of digastric, tensor tyMpani, anterior 2/3 of tongu Nerves: - CN V2 (Maxillary branch) and V3 (Mandibular) ("chew")

Question 294

Which cartilage, muscles and nerves does the 2nd pharyngeal arch give rise to?

Answer 294

All of the "S's" Cartilage: - Reichert cartilage: Stapes, Styloid process, lesser horn of hyoid, Stylohyoid ligament Muscles: - Muscles of facial expreSSion, Stapedius, Stylohyoid, platySma, posterior belly of digastric Nerves: - CN VII (S = seven) (facial expreSSion) Smile

Question 295

Which cartilage, muscles and nerves does the 3rd pharyngeal arch give rise to?

Answer 295

Cartilage: - Greater horn of hyoid Muscles: - Stylopharyngeus (think of styloPHARYNGEus innervated by glossoPHARYNGEalnerve) Nerve: - CN IX (STYLo-pharyngeus)swallow STYLishly

Question 296

Which cartilage, muscles and nerves do the 4th and 6th pharyngeal arches give rise to?

Answer 296

Cartilage: - Arytenoids, Cricoid, Corniculate, Cuneiform, Thyroid (used to sing and ACCCT) Muscles: - 4th arch: most pharyngeal constrictors; cricothyroid, levator veli palatini - 6th arch: all intrinsic muscles of larynx except cricothyroid Nerves: - 4th arch: CN X (superior laryngeal branch) simply swallow - 6th arch: CN X (recurrent laryngeal branch) speak

Question 297

What is necrolytic migratory erythema, and which neuroendocrine tumor is it classically seen in?

Answer 297

Necrolytic migratory erythema is a characteristic skin finding, identified by the pruritic and painful, small erythematous plaques typically affecting the face, perineum, and extremities, in patients with a glucagonoma.

Question 298

Which acid suppressing agent used to treat GERD, has unfortunate anti-androgenic side effects? Which drug, with the same MOA can ben given instead?

Answer 298

Cimetidine (an H2 blocker) can cause decreased libido, gynecomastia, and impotence. Ranitidine, is also an H2 blocker but doesn't have antiandrogenic side effects.

Question 299

What is the mechanism of contraction bands seen on histology following an acute MI?

Answer 299

Contraction bands can be seen within the first 24 hours of an MI, in the setting of reperfusion injury which generates free radicals and causes hypercontraction of myofibrils through increased free calcium reflux.

Question 300

Which mnemonic describes the presentation of von Hippel-Lindau disease?

Answer 300

HARP: - Hemangioblastomas (often described as highly vascular with hyperchromatic nuclei) - Angiomatosis - Renal cell carcinomas (bilateral) - Pheochromocytomas

Question 301

How can sarcodosis leader to hypervitaminosis D and hypercalcemia?

Answer 301

The macrophages responsible for the non-caseating granulomas contain 1α-hydroxylase activity, which converts Vitamin D to the active form and subsequently causes calcium reabsorption.

Question 302

Medullary thyroid cancer is a malignancy of which cells? What are these cells derived from?

Answer 302

Medullary thyroid cancer = malignancy of the parafollicular C cells These cells are derived from neural crest cells

Question 303

What is the name of the gene regulated by PPAR-γ that is responsible for increasing insulin sensitivity when treated by a glitazone drug?

Answer 303

Adiponectin

Question 304

Which lipid-lowering class is the most effective at treating hypertriglyceridemia? What is the MOA?

Answer 304

The fibrates Fibrates upregular PPAR-alpha and increase the transcription of lipoprotein lipase, which increases the degradation of TGs circulating in chylomicrons and VLDL

Question 305

What is the single-most preventable cause of death in the United States?

Answer 305

Smoking/tobacco cessation

Question 306

Patient presenting with hypogonadism, anosmia and delayed puberty most likely has what?

Answer 306

Kallmann syndrome - an absence of GnRH secretory neurons in the hypothalamus due to defective migration from the olfactory placode.

Question 307

What is the MOA of ezetimibe?

Answer 307

Ezetimibe prevents cholesterol absorption at small intestine brush border.

Question 308

Describe the mechanism of pretibial myxedema in Graves disease:

Answer 308

Antibodies against the thyrotropin receptor causes an accumulation of glycosaminoglycans and adipogenesis.

Question 309

Which ribs overlie the spleen?

Answer 309

The 9th, 10th, and 11th left ribs.

Question 310

Congenital diaphragmatic hernia often leads to death due to:

Answer 310

Pulmonary hypoplasia (caused by compression of the developing lung buds).

Question 311

What is the most important lecithin that composes pulmonary surfactant?

Answer 311

Dipalmitoylphosphatidylcholin (DPPC).

Question 312

What mnemonic can be used to remember thoracic level at which the IVC, esophagus, and aorta penetrate the diaphragm?

Answer 312

"I (8), 10 Eggs At 12" - IVC -->T8 - Esophagus --> T10 - Aorta --> T12

Question 313

What is the equation for determining physiologic dead space

Answer 313

Vd = Tv x (PaCO2 - PeCO2)/PaCO2 - Vd = physiologic dead space (in mL) - Tv = tidal volume (in mL) - PaCO2 = arterial PCO2 - i.e. how much CO2 is going into the lungs - PeCO2 = expired PCO2 - i.e. how much CO2 is exiting out of the lungs after gas exchange Therefore, the closer the PeCO2 is to the PaCO2, the more efficient the gas exchange was.

Question 314

How can you distinguish methemoblobinemia from carboxyhemoglobin?

Answer 314

Methemoglobinemia: cyanosis and chocolate-brown blood; due to oxidized form of Hb (Fe2+ to Fe3+; caused by nitrites, dapsone, etc.) which does not bind O2 as readily, but has increased affinity for cyanide. Carboxyhemoblobinemia: bright read blood, headaches, cherry-red lips (cherry red is dead); CO binds competitively to Hb and with 200x greater affinity than O2.

Question 315

What is the effect of CO on the Hb-dissociation curve?

Answer 315

Causes a left shift and reduces O2 binding capacity

Question 316

Aspiration of a large foreign body is more likely to occur where?

Answer 316

Right mainstem bronchus

Question 317

Epistaxis originating in which part of the nose and via which artery can cause a life-threatening hemorrhage?

Answer 317

A posterior segment bleed, due to a bleed in the sphenopalatine artery.

Question 318

What is the most common cause of pulmonary embolism?

Answer 318

DVT

Question 319

What is Virchow's triad of DVT?

Answer 319

SHE: - Stasis (e.g. car ride, flight) - Hypercoagulability (e.g. high-estrogen states, defect in coagulation cascade proteins) - Endothelial damage

Question 320

Describe the time-course for a diagnosis of chronic bronchitis?

Answer 320

Must have a productive cough for > 3months in a year for > 2 consecutive years

Question 321

What is the Reid index?

Answer 321

The Reid index is a measure of mucosal thickness to the thickness of the wall between the bronchial eipthelium and cartilage. Someone with chronic bronchitis (i.e. hypertrophy and hyperplasia of mucus-secreting glands) will have a Reid index >50%

Question 322

Which two lung diseases is silicosis associated with?

Answer 322

Bronchogenic carcinoma and TB

Question 323

What is Pickwickian Syndrome?

Answer 323

A synonym for obesity hypoventilation syndrome.

Question 324

What is the natural progression of lobar pneumonia?

Answer 324

CRGR: - Congestion (Day 1-2): Red-purple with mostly bacteria - Red hepatization (Day 3-4): Red-brown, with fibirn, bacteria, WBCs and RBCs - Gray hepatization (Day 5-7): uniformly gray, mostly full of WBCs and fibrin - Resolution (Day 8+): enzymes digest components of exudate

Question 325

What are the 5 major complications of lung cancer (SPHERE)?

Answer 325

SPHERE: - SVC syndrome - Pancoast tumor - Horner syndrome - Endocrine (paraneoplastic) - Recurrent laryngeal nerve compression (hoarsness) - Effusions (pleural or pericardial)

Question 326

Which lung cancer is considered a neoplasm of Kulchitsky cells and stains for Chromogranin A?

Answer 326

Small cell carcinoma | - Kulchitsky cells = small dark blue cells

Question 327

Which lung cancer will stain positive for mucin?

Answer 327

Adenocarcinoma (remember "adeno" = mucus glands)

Question 328

Which lung cancer produces PTHrP; how will it present?

Answer 328

Squamous cell | - PTHrP production leads to hypercalcemia

Question 329

Which lung cancer has an excellent prognosis, and what are its findings?

Answer 329

Bronchial carcinoid tumor has an excellent prognosis, and rare metastases. - Can present with carcinoid syndrome (flushing, diarrhea, wheezing) - Nest (or rosettes) of neuroendocrine cells can be seen on histology

Question 330

Why are 2nd generation antihistamines less sedating than 1st generation?

Answer 330

2nd generation has less CNS entry/penetration

Question 331

What is the mechanism of action of N-acetylcysteine as a mucolytic?

Answer 331

N-acetylCYSTEINE disrupts disulfide bonds (made of 2 CYSTEINE residues)

Question 332

What is the use and MOA of bosentan?

Answer 332

BosENtan is a competitively antagonist of the ENdothelin-1 receptor, it is used to treat pulmonary hypertension. ADR: hepatotoxic

Question 333

Why do patient's with emphysema exhibit pursed-lip breathing?

Answer 333

Exhaling through pursed lips increases the pressure in the alveoli and prevents them from collapsing.

Question 334

What is the histopathologic description of granulomas found in TB?

Answer 334

TB granulomas are composed of epithelioid cells (macrophages with abundant pink cytoplasm) with surrounding multinucleated giant cells and lymphocytes

Question 335

Which lung pathology has a honeycombing appearance on CXR and often presents with clubbing of the nails?

Answer 335

Idiopathic pulmonary fibrosis (restrictive lung disease)

Question 336

How does a positive skew differ from a negative skew?

Answer 336

The direction of the "skew" is determined by the outliers of the set. Positive skew: high outliers cause the mean to be greater than the median (tail is to the right) Negative skew: low outliers cause the mean to be less than the median (tail is to the left).

Question 337

What is the most commonly recommended treatment for TB prophylaxis and LTBI monotheraphy?

Answer 337

INH + pyridoxine (B6) X 6months

Question 338

A child with recurrent infections is presenting with absent heart sounds in the mitral, tricuspid and pulmonary position, but a normal sound in the aortic position. Which lung pathology is this patient at increased risk for?

Answer 338

This child has Kartagener's (indicated by the recurrent infections and suggestion of situs inversus) and is at increased risk for bronchiectasis.

Question 339

Which two enzymes are produced by the macrophages in sarcoidosis, and can therefore be used as markers?

Answer 339

1alpha-hydroxylase and ACE

Question 340

How can osteogenesis imperfecta be distinguished from child abuse?

Answer 340

OI will generally involve a history of fractures from benign incidents (i.e. standing height) Abuse will involved fractures (eg, ribs, long bone spiral, multiple in different stages of healing), bruises (eg, trunk, ear, neck; in pattern of implement), burns (eg, cigarette, buttocks/thighs), subdural hematomas, retinal hemorrhages. During exam, children often avoid eye contact.

Question 341

What gives elastin its elastic properties?

Answer 341

Cross-linking that takes place between lysine residues.

Question 342

Which interleukin is chemotactic for eosinophils?

Answer 342

IL-5

Question 343

What is most recommended therapy for thromboembolic therapy in pregnant women?

Answer 343

Low molecular-weight heparins (e.g. enoxaparin) are recommended because they don't cross the placenta and therefore don't carry a high risk of bleeding.

Question 344

What does the truncus arteriosus give rise to?

Answer 344

Ascending aorta and pulmonary trunk

Question 345

What does the bulbus cordis give rise to?

Answer 345

Smooth outflow tracts of the left and right ventricles

Question 346

What does the endocardial cushion give rise to?

Answer 346

Atrial septum and membranous interventricular septum

Question 347

What does the primitive pulmonary vein give rise to?

Answer 347

Smooth part of left atrium

Question 348

What does the left horn of the sinus venosus give rise to?

Answer 348

Coronary sinus

Question 349

What does the right horn of the sinus venosus give rise to?

Answer 349

Smooth part of right atrium

Question 350

What do the right common cardinal vein and right anterior cardinal veins give rise to?

Answer 350

The SVC

Question 351

Kartagener's syndrome causes situs inversus due to defects in which protein?

Answer 351

Left-to-right dynein

Question 352

What is the first functional organ in vertebrate organisms and when does it begin?

Answer 352

The heart | - week 4 of development

Question 353

Describe the process of atrial septation:

Answer 353

1. Septum primium begins superiorly, makes its way down toward the endocardial cushion and then pauses to form foramen primium. As the septum primium continues down, it closes the foramen primium and forms the foramen secundum 2. The septum secundum then develops to the right (patient's right) of the septum prim and begins to expand down to cover most, if not all of the first septum to form the foramen secundum and allow the R --> L

Question 354

What are the three important fetal cardiac shunts and what are their functions?

Answer 354

1. Ductus venosus - 2/3 of the oxygenated blood coming in from the umbilical vein the bypasses the fetal liver and goes straight to the RA via the IVC where in can be directed straight to systemic circulation via the FO: 2. Foramen ovale - a R-->L shunt in which oxygenated blood in the RA goes straight to the LA where it can then be pumped by the LV into systemic circulation (esp. to the head). 3. Ductus arteriosus - deoxygenated blood that came in via the SVC goes through the LV and enters the pulmonary trunk, but instead of going to the lung, it is shunted to the aorta (after the main stems) where it descends down the thoracic/abdominal aorta before returning to the umbilical arteries where it is returned to mom.

Question 355

What does the allantois become?

Answer 355

MediaN umbilical ligament

Question 356

Foramen ovale becomes -->

Answer 356

Fossa ovalis

Question 357

Notochord becomes -->

Answer 357

Nucleus pulposus (spinal cord discs --> can impinge and cause nerve damage)

Question 358

UmbiLical arterials become -->

Answer 358

MediaL umbilical ligaments

Question 359

Which part of the heart has dual blood supply from two major coronary arteries?

Answer 359

The anterolateral papillary muscle (receives blood from the LCX and LAD)

Question 360

Explain the physiology behind a wide split:

Answer 360

Wide splitting occurs when there is any cadiac etiology that delays the emptying of the RV (therefore delaying closure of the pulmonic valve): - pulmonic stenosis - RBB - the LV contracts before the RV

Question 361

What causes a fixed split?

Answer 361

Fixed splitting is when the second heart sound is split (i.e. A2 and P2)the same distance on both inspiration and expiration (because RV is increased throughout both systole and diastole) --> most commonly due to ASD.

Question 362

What a mid-systolic click most diagnostic for?

Answer 362

Mitral valve prolapse - Mid-systolic click followed by a crescendo systolic murmur - Often occurs due to myxomatous degeneration

Question 363

How can you distinguish between a holosystolic murmur caused by tricuspid regurgitation vs. VSD?

Answer 363

The tricuspid regurgitation murmur will radiate to the axilla.

Question 364

Blowing murmur during early diastole?

Answer 364

Aortic regurgitation

Question 365

Which murmur occurs continuously (i.e. over both systole and diastole)?

Answer 365

PDA (because blood will always be flowing through the PDA no matter what phase you're in)

Question 366

Where is the SA node located?

Answer 366

SA node is located near the SVC

Question 367

Where is the AV node located?

Answer 367

AV node is located in the posteroinferior part of the interatrial septum (near tricuspid orifice)

Question 368

What are the conduction speeds of the heart?

Answer 368

- AV: 0.05 m/sec - Ventricle: 0.3 m/sec - Atria: 1.1 m/sec - Purkinje: 2.2 m/sec

Question 369

What is the treatment for Torsades de pointes?

Answer 369

Magnesium sulfate

Question 370

What is the difference between Romano-Ward syndrome and Jervell-Lange-Nielsen syndrome?

Answer 370

- Romano-WarD is autosomal Dominant, and purely cardiac (pure heart like the ROMAN Catholics) - Jervell and Lange-Nielsen syndrome - autosomal recessive and is accompanied by sensorineural deafness (longer name, harder to hear)

Question 371

What is Brugada syndrome?

Answer 371

Brugada syndrome is an autosomal dominant disorder (mostly in Asain males - Brugada is a Japanese surname). Has an ECG pattern of pseudo-RBBB due to a defect in the sodium channel that prevents both ventricles from depolarizing simultaneously.

Question 372

Delta waves and a widened QRS likely indicate:

Answer 372

Wolff-Parkinson-White syndrome

Question 373

Absent P waves with irregularly irregular spacing of QRS complexes?

Answer 373

Atrial fibrillation

Question 374

4:1 sawtooth pattern with regular spacing and discernible P waves

Answer 374

Atrial flutter

Question 375

No discernible rhythm at all (no P wave, no PR interval, no QRS)

Answer 375

Ventricular fibrillation! EMERGENCY - not compatible with life without immediate CPR and defibrillation

Question 376

Which AV block is characterized by a prolonged PR interval?

Answer 376

First degree | "If the R is far from P then you have a 1st degree"

Question 377

Which AV block is characterized by increasingly prolonged PR intervals with a sudden absence of QRS interval?

Answer 377

Second degree - Mobitz Type 1: Wenckebach | "Longer, longer, longer drop then you have a Wenckebach"

Question 378

Which AV block is characterized by dropped beats that are not preceded by change in the length of the PR interval?

Answer 378

Second degree - Mobitz Type 2 | "If only some P's don't get through, then you have Mobitz Type 2"

Question 379

Which AV block is characterized by a complete lack of coordination between atrial and ventricular contraction?

Answer 379

``` Third Degree (Complete) "It P's and Q's don't agree, then you have a 3rd degree" ```

Question 380

Which disease is associated with a 3rd degree AV block?

Answer 380

Lyme disease

Question 381

True or false, central chemoreceptors can respond to pO2?

Answer 381

False! O2 can't cross the BBB

Question 382

What are the normal cardiac pressures?

Answer 382

``` RA: 5 mmHg RV: 25/5 mmHg Pulmonary artery: 25/10 mmHg PCWP: 4-12 mmHg LA: <12 mmHg LV: 130/10 mmHg Aorta: 130/90 ```

Question 383

What are the T's associated with early cyanosis?

Answer 383

- Truncus arteriosus (1 vessel) - Transposition of great vessels (2 vessels switch) - Tricuspid atresia (3 = tri) - Tetralogy of Fallot (4 = tetra) - TAPVR (5 letters in name) - total anomalous pulmonary venous return

Question 384

What causes rib notching which occurs with coarctation of the aorta is caused by?

Answer 384

By blocking the normal flow out of the aorta, collaterals are accessed via the intercostal arteries. The intercostals are locating on the underside of the ribs, which results in a "notched" appearnace on Xray due to the dilation of these arteries producing an indentation.

Question 385

What is the pathophysiology of heart defects seen in congenital rubella?

Answer 385

Viral proliferation effects the normal fusion/activity of the cardiac myocytes, smooth muscle and endothelium. This results in a failure of closure of various tissues. - PDA, PA stenosis, septal defects

Question 386

What is the pathophysiology of heart defects seen in Down Syndrome?

Answer 386

Down Syndrome results in endocardial cushion defects: | - AV septal defect, VSD, ASD

Question 387

An which two pathologies are associated with maternal diabetes?

Answer 387

- Transposition of great vessels | - Caudal regression syndrome

Question 388

Williams syndrome features what cardiac anomaly?

Answer 388

Remember, WilliAmS syndrome features AS (aortic stenosis) due to elastic defect resulting in a stenotic/stiff aortic valve.

Question 389

What is fibromuscular dysplasia?

Answer 389

A strange occurence of high-blood pressure (usu. in a healthy, YA woman) --> due to genetic defect that causes the tunica media to develop invaginations narrowing the lumen - beads on a string

Question 390

Pipe stem arteries on xray indicate what pathology?

Answer 390

Mönckeberg sclerosis (medial calcific sclerosis) - doesn't obstruct blood flow because the intima are not involved

Question 391

Sudden, tearing chest pain that radiates to the back is most likely?

Answer 391

Aortic dissection

Question 392

Which aortic dissection, Type A or Type B has a greater risk of cardiac tamponade, and why?

Answer 392

Type A - because it is ABOVE the ligamentum arteriosum and can therefore leak into the pericardial space

Question 393

What type of cardiomyopathy is associated with Friedreich Ataxia?

Answer 393

HCOM

Question 394

Are most cardiac tumors primary or secondary (due to metastases)? What are the most common causes, regardless?

Answer 394

Most cardiac tumors are due to metastases (melanoma = #1) The most common primary cardiac tumor in adults is a myxoma. The most common primary cardiac tumor in children is a rhabdomyoma.

Question 395

What is the diagnosis? | - Elderly patient with a temporal artery headache, and flu-like symptoms with widespread joint and muscle pain?

Answer 395

Giant cell (temporal) arteritis, characterized by focal granulomatous inflammation (resulting in "giant cell").

Question 396

Which two anti-arrhythmic drugs can cause a lupus-like syndrome?

Answer 396

Procainamide and hydralazine | anti-histone Abs

Question 397

Which structures are derived from the 1st aortic arch?

Answer 397

Maxillary artery (1st arch is MAXimal)

Question 398

Which structures are derived from the 2nd aortic arch?

Answer 398

Stapedial artery and hyoid artery (Second = Stapedial)

Question 399

Which structures are derived from the 3rd aortic arch?

Answer 399

Common Carotid artery and proximal internal Carotid (C = 3rd letter of alphabet)

Question 400

Which structures are derived from the 4th aortic arch?

Answer 400

LARS: - Left --> aortic arch - Right --> subclavian artery

Question 401

Which structures are derived from the 6th aortic arch?

Answer 401

Proximal part of pulmonary arteries and ductus arteriosus

Question 402

Which arteritis is associated with and increased ESR, granulomatous thickening and narrowing of aortic arch and proximal great vessels?

Answer 402

Takayasu Arteritis ("pulseless disease")

Question 403

What are the findings in Kawasaki's disease?

Answer 403

Usually affects Asian children <4yo. The symptoms can be remembered by "CRASH and BURN" Asian children < 4 years old. - Conjunctival injection, - Rash (polymorphous desquamating) - Adenopathy (cervical) - Strawberry tongue (oral mucositis) - Hand-foot changes (edema, erythema) - Fever (Burn) These patients are also at an increased risk for coronoary artery aneurysms which can thrombose and rupture. - Therefore they are the only pediatric patients in which taking aspirin with a fever is not contraindicated (not a risk for Reye's syndrome)

Question 404

Following an acute MI, when do Troponin I levels become elevated and how long do they remain elevated?

Answer 404

Troponin I levels first become elevated 4 hours after an MI, and remain elevated for 7-10 days.

Question 405

Following an acute MI, when do CK-MB levels become elevated and how long do they remain elevated?

Answer 405

The CK-MB fraction level increases 6–12 hours after an MI and peaks within the first 24 hours. It is used to detect a subsequent MI, or reinfarction, but is not used to diagnose an initial MI.

Question 406

Which vasculitis is features transmural inflammation of the arterial wall with fibrinoid necrosis, and may involve different stages of inflammation coexisting in different vessels?

Answer 406

Polyarteritis nodosa | - Hepatitis B seropositivity is found in 30% of patients

Question 407

Which neurocutaneous syndrome is associated with childhood cardiac rhabdomyomas?

Answer 407

``` Tuberous sclerosis (HAMARTOMAS) Hamartomas in CNS and skin Angiofibromas Mitral regurgitation Ash-leaf spots Rhabdomyoma (Tuberous sclerosis) autosomal dOminant; Mental retardation (intellectual disability); Angiomyolipoma (renal) Seizures, Shagreen patches. ```

Question 408

What is the minimum cutoff value for stable angina?

Answer 408

> 70% occlusion

Question 409

What is an Aschoff body?

Answer 409

An Aschoff body is a granuloma with giant cells, that are seen in acute bacterial rheumatic fever. They may often contain Anitschkow cells (enlarged macrophages with ovoid, wavy rod-like nucleus).

Question 410

What is the best indicator for the severity of mitral stenosis?

Answer 410

The length of time between S2 and the opening snap. (Shorter S2-OS interval = worse)

Question 411

What type of collagen composes the scar tissue that is observed weeks to months following an MI?

Answer 411

Type 1 collagen (MI = M1)

Question 412

What is the intrapleural pressure at FRC?

Answer 412

-5 cm H2O

Question 413

What is the most common CF mutation?

Answer 413

The ∆F508 is the most common cystic fibrosis mutation, a 3-base pair mutation in the CFTR gene in which there is a deletion in Phe, causing impaired post-translational processing (improper folding). This abnormal protein is detected by the ER and sent to the proteasome for degradation, so it never reaches the cell surface.

Question 414

Which genus of bacteria lack a cell wall?

Answer 414

Mycoplasma

Question 415

Which personality disorder often features the immature defense mechanism of splitting?

Answer 415

Borderline personality disorder

Question 416

At what time/duration of depriving infants of affection, does irreversible damage result?

Answer 416

Deprivation for > 6months

Question 417

What is the most common form of child maltreatment?

Answer 417

Child neglect

Question 418

What is the order that a patient experiencing delerium loses orientation?

Answer 418

(First) time > place > person (last)

Question 419

What is Korsakoff syndrome and what are its features?

Answer 419

Korsakoff syndrome occurs in the setting of severe B1 deficiency (part of Wernicke's), due to destruction of the mammillary bodies. Features: - anterograde amnesia - confabulations ("konfabulations")

Question 420

What are some reversible causes of dementia?

Answer 420

- Depression (pseudodementia) - Hypothyroidism - Vitamin deficiency (esp. B1, B3, B12) - Normal pressure hydrocephalus

Question 421

How is schizoaffective disorder differentiated from a major mood disorder with psychotic features?

Answer 421

Schizoaffective disorder = criteria met for both schizophrenia and a major mood disorder (e.g. the patient can have both schizophrenia and have bipolar disorder); however, the main difference is that schizoaffective disorder must have a period >2weeks of hallucinations or delusions on their own, without a major mood episode

Question 422

What criteria can be used to diagnose a manic episode?

Answer 422

DIGFAST: 3 or more of the following present for at last 1 week: - Distractibility - Irresponsibility—seeks pleasure without regard to consequences (hedonistic) - Grandiosity—inflated self-esteem - Flight of ideas—racing thoughts - Increase in goal-directed Activity/psychomotor Agitation - need for Sleep decreases - Talkativeness or pressured speech

Question 423

Which personality disorder can be treated/managed with dialectical behavior therapy?

Answer 423

Borderline

Question 424

How does a sleep terror differ from a nightmare?

Answer 424

Sleep terrors involve sudden screaming in the middle of the night and occur during Slow-wave sleep and there is generally no memory of the arousal episode upon waking. NightMares occur during REM sleep, and are remembered upon waking.

Question 425

What neurotransmitter abnormality leads to narcolepsy/cataplexy?

Answer 425

Narcolepsy is caused by decreased production of hypocretin(orexin) in the lateral hypothalamus (normally responsible for preventing early entry into REM sleep)

Question 426

What are the general signs of depressant intoxication and withdrawal:

Answer 426

Intoxication: mood elevation, decreased anxiety, sedation, respiratory depression Nonspecific (think about it...you're taking away GABA/inhibition): anxiety, tremor, seizures, insomnia

Question 427

What are the specific signs and symptoms of alcohol intoxication and withdrawal?

Answer 427

Intoxication: Emotional lability, slurred speech, ataxia, coma, blackouts. Serum γ-glutamyltransferase (GGT)—sensitive indicator of alcohol use. AST = 2x ALT value Withdrawal: Time from last drink: 3–36 hr: minor symptoms similar to other depressants 6–48 hr: withdrawal seizures 12–48 hr: alcoholic hallucinosis (usually visual) 48–96 hr: delirium tremens (DTs) in 5% of cases Treatment: benzodiazepines.

Question 428

What are the specific signs and symptoms of opiate intoxication and withdrawal?

Answer 428

Intoxication: Euphoria, respiratory and CNS depression, gag reflex, pupillary constriction (pinpoint pupils), seizures (overdose). Most common cause of drug overdose death. Treatment: naloxone Withdrawal: Sweating, dilated pupils, piloerection (“cold turkey”), fever, rhinorrhea, yawning, nausea, stomach cramps, diarrhea (“flu-like” symptoms). Treatment: long-term support, methadone, buprenorphine

Question 429

What are the specific signs and symptoms of barbiturate intoxication and withdrawal?

Answer 429

Intoxication: Low safety margin, marked respiratory depression Withdrawal: Delirium, life-threatening cardiovascular collapse

Question 430

What are the specific signs and symptoms of benzodiazepine intoxication and withdrawal?

Answer 430

Intoxication: Greater safety margin. Ataxia, minor respiratory depression. Treatment: flumazenil Withdrawal: Sleep disturbance, depression, rebound anxiety, seizure.

Question 431

What are the general signs of stimulant intoxication and withdrawal?

Answer 431

Intoxication: mood elevation, psychomotor agitation, insomnia, cardiac arrhythmias, tachycardia, anxiety Withdrawal: post-use “crash,” including depression, lethargy, appetite, sleep disturbance, vivid nightmares

Question 432

What are the specific signs and symptoms of amphetamine intoxication and withdrawal?

Answer 432

Effects of intoxication last for hours: Euphoria, grandiosity, pupillary dilation, prolonged wakefulness and attention, hypertension, tachycardia, anorexia, paranoia, fever. Severe: cardiac arrest, seizures. Treatment: benzodiazepines for agitation and seizures

Question 433

What are the specific signs and symptoms of cocaine intoxication and withdrawal?

Answer 433

Effects don't last as long as amphetamines: Impaired judgment, pupillary dilation, hallucinations (including tactile), paranoid ideations, angina, sudden cardiac death. Treatment: α-blockers, benzodiazepines. ß-blockers not recommended

Question 434

What are the specific signs and symptoms of caffeine intoxication and withdrawal?

Answer 434

Intoxication: Restlessness, diuresis, muscle twitching. Withdrawal: Headache, difficulty concentrating, flu-like symptoms

Question 435

What are the specific signs and symptoms of nicotine intoxication and withdrawal?

Answer 435

Intoxication: Restlessness Withdrawal: Irritability, anxiety, restlessness, difficulty concentrating. Treatment: nicotine patch, gum, or lozenges; bupropion/varenicline

Question 436

What are the specific signs and symptoms of LSD intoxication and withdrawal?

Answer 436

Perceptual distortion (visual, auditory), depersonalization, anxiety, paranoia, psychosis, possible flashbacks]- NOT habit forming

Question 437

What are the specific signs and symptoms of marijuana intoxication and withdrawal?

Answer 437

Intoxication: Euphoria, anxiety, paranoid delusions, perception of slowed time, impaired judgment, social withdrawal, increased appetite, dry mouth, conjunctival injection, hallucinations. Pharmaceutical form is dronabinol: used as antiemetic (chemotherapy) and appetite stimulant (in AIDS) Withdrawal: Irritability, anxiety, depression, insomnia, restlessness, decreased appetite

Question 438

What are the specific signs and symptoms of MDMA (ecstasy) intoxication and withdrawal?

Answer 438

Intoxication: euphoria, disinhibition, hyperactivity, distorted sensory and time perception, teeth clenching. Life-threatening effects include hypertension, tachycardia, hyperthermia, hyponatremia (via diaphoresis, SIADH and water intoxication from users replacing fluid due to dehydration from sweating). Withdrawal: Depression, fatigue, change in appetite, difficulty concentrating, anxiety.

Question 439

What are the specific signs and symptoms of phencyclidine (PCP) intoxication and withdrawal?

Answer 439

Intoxication: Violence (super strong and can't feel pain), impulsivity, psychomotor agitation, nystagmus (BUZZWORD), tachycardia, hypsertension, analgesia, psychosis, delirium, seizures. Trauma is most common complication. Treatment: benzodiazepines, rapid-acting antipsychotic

Question 440

Which drug should be given to a patient experiencing acute alcohol withdrawal?

Answer 440

Benzodiazepines (specifically oxazepam, lorazepm or temazepam because these drugs undergo Phase II metabolism and are therefore not contraindicated in someone with liver disease)

Question 441

What embryologic structure is the neural tube derived from?

Answer 441

The Neural plate/folds

Question 442

What does the notochord give rise to?

Answer 442

The Nucleus pulposus of intervertebral discs

Question 443

Which primary vesicle is the telencephalon derived from, and what are the adult wall/cavitary derivatives that it gives rise to?

Answer 443

Forebrain --> Telencephalon --> Walls: cerebral hemispheres and cavities: lateral ventricles

Question 444

Which primary vesicle is the diencephalon derived from, and what are the adult wall/cavitary derivatives that it gives rise to?

Answer 444

Forebrain --> Diencephalon --> Walls: thalamus, hypothalamus and cavities: third ventricle

Question 445

Which primary vesicle is the mesencephalon derived from, and what are the adult wall/cavitary derivatives that it gives rise to?

Answer 445

Midbrain --> Mesencephalon --> Walls: midbrain and cavities: aqueduct

Question 446

Which primary vesicle is the metencephalon derived from, and what are the adult wall/cavitary derivatives that it gives rise to?

Answer 446

Hindbrain --> Metencephalon --> Walls: pons, cerebellum and cavities: upper part of fourth ventricle

Question 447

Which primary vesicle is the myelencephalon derived from, and what are the adult wall/cavitary derivatives that it gives rise to?

Answer 447

Hindbrain --> Myelencephalon --> Walls: medulla, and cavities: lower part of fourth ventricle

Question 448

Which two serum markers can be used to diagnose NTDs?

Answer 448

ACHE and AFP

Question 449

What is the diagnosis? This patient is presenting with loss of pain and temperature sensation in a cape-like distribution, with preserved position and vibration sense. MRI also reveals a cystic cavity within the central canal of the spinal cord.

Answer 449

Syringomyelia

Question 450

What are some of the functions and features of astrocytes?

Answer 450

- Provide physical and repair support to neurons - Remove excess neurotransmitter - Component of the BBB - Contributes to reactive gliosis in response to neural injury - Marker: GFAP - Derived from neuroectoderm

Question 451

What are the function of microglia? How are microglia unique compared to the rest of the CNS?

Answer 451

Phagocyte scavenger cells. They are derived from mesoderm.

Question 452

Which kinetic constant does myelin increase to increase the conduction velocity of an axon?

Answer 452

The space constant (aka. the length constant - the distance that a graded electric potential will travel along a nemuron via passive electrical conduction before requiring a second depolarization)

Question 453

What are the difference between the C fibers and the Aδ fibers in free nerve endings?

Answer 453

C = slow, unmyelinated --> cold/hot Aδ = fast, myelinated --> pAin

Question 454

What is the function of Meissner corpuscles?

Answer 454

Found on hairleSS skin; soft "s's" = soft/light touch

Question 455

What is the function of Pacinian corpuscles?

Answer 455

Pressure --> felt in deep skin/ligaments/joints

Question 456

What is the function of Merkel discs?

Answer 456

hard "k" is SHARP like shapes/edges

Question 457

What is the function of Ruffini corpuscles?

Answer 457

Can feel objects with a ruff(rough) surface won't slip from the skin

Question 458

Where is acetylcholine synthesized?

Answer 458

Basal nucleus of Meynert

Question 459

Where is dopamine synthesized?

Answer 459

Ventral tegmentum, SNc (think PD)

Question 460

Where is GABA synthesized?

Answer 460

Nucleus accumbens

Question 461

Where is NE synthesized?

Answer 461

Locus cerulus

Question 462

Where is 5-HT synghtesized?

Answer 462

Raphe nucleus

Question 463

Which part of the brain lacks a BBB?

Answer 463

Area postrema the "vomiting center" - this is why chemo drugs cause vomiting

Question 464

What are the stages of sleep and which wave forms are seen in each?

Answer 464

At night "BATS Drink Blood" - Awake (eyes open) = Beta waves - highest freq, lowest amplitude - Awake (eyes closed) = Alpha waves - Stage N1 = Theta waves (5%) - light sleep - Stag N2 = Sleep spindles/K complexes (45%) - deep sleep where bruxism (pronounce bruKxism) occurs - Stage N3 = Delta (25%) - deepest non-REM, when sleepwalking, night terrors and bedwetting occur REM - REM sleep = Beta (25%) - loss of motor ton, variable pulse and blood pressure driven by ACh

Question 465

What is the equation for the cerebral perfusion pressure?

Answer 465

CPP = MAP - ICP

Question 466

Which structures pass through the cribriform plate?

Answer 466

CN1 (duh...olfactory nerve!)

Question 467

Which structures pass through the optic canal?

Answer 467

CNII and ophthalmic artery (makes sense—optic canal leads to the eye)

Question 468

Which structures pass through the Superior orbital fissure?

Answer 468

CNIII, CNIV, CN VI (i.e. all of the extraocular muscle nerves and CN V1 - the ophthalmic branch of trigeminal

Question 469

Which structures pass through the foramen rotundum?

Answer 469

CN V2 - the maxillary branch of trigeminal

Question 470

Which structures pass through the the foramen ovale?

Answer 470

CN V3 - the mandibular branch of trigeminal

Question 471

Which structures pass through the foramen spinosum?

Answer 471

Middle meningeal artery

Question 472

Which structures pass through the internal auditory meatus?

Answer 472

CNVII and CN VIII

Question 473

Which structures pass through the jugular forament?

Answer 473

CN IX, X, XI and the jugular vein

Question 474

Which structures pass through the hypoglassal canal

Answer 474

the HYPOGLOSSAL nerve (CNXII)

Question 475

Which structures pass through the foramen magnum?

Answer 475

Brainstem, spinal root of CNXI, vertebral arteries.

Question 476

Where is the needle most safely placed for lumbar puncture?

Answer 476

Between L3 and L4 or L4 and L5 | "L3 and L5 to keep the cord alive!"

Question 477

What is the time course for the various changes in ischemic brain disease?

Answer 477

12-24 hours - red neurons (eosinophilic cytoplasm with pyknotic nuclei and loss of Nissl substance) 24-72 hours - necrosis and neutrophils 3-5 days - Macrophages (microglia) 1-2 weeks - Reactive gliosis + vascular proliferation > 2 weeks - glial scar (around cystic space)

Question 478

Rupture of which blood vessels results in a subdural hematoma?

Answer 478

Bridging veins

Question 479

What should a subarachnoid hemorrhage be treated with?

Answer 479

CCB to prevent vasospasm

Question 480

What is a Charcot-Bouchard aneurysm?

Answer 480

A type of intraparenchymal hemorrhage that occurs in the basal ganglia or internal capsule, usually due to a microaneurysm of lenticulostriate vessels.

Question 481

What are the clinical manifestations of medial medullary syndrome and which artery is affected?

Answer 481

Anterior spinal artery - duh...the ASA lies along the medulla - contralateral paralysis (motor tract decussates here) - contralateral proprioception decrease (DCML decussats here) - ipsilateral hypoglossal dysfunction (CN12 is located right there!)

Question 482

What are the clinical manifestations of lateral medullary (Wallenberg) syndrome and which artery is affected?

Answer 482

Posterior inferior cerebellar artery - duh..the PICA is located off to the lateral medulla, which would affect the nerves of the nucleus ambiguus (IX, X, XI) "Don't PICA horse (hoarseness) that can't eat (dysphagia) - decreased pain and temp sensation from contralateral body/ipsilateral face

Question 483

What are the clinical manifestations of lateral pontine syndrome and which artery is affected?

Answer 483

Anterior inferior cerebellar artery - duh...the AICA sits on the lateral pons "Facial droop means AICA's pooped" - paralysis of face (decreased lacrimation, salivation, and decreased tates from anterior 2/3 of tongue) - ipsilateral Horner syndrome

Question 484

What are the clinical manifestations of "Locked-in" syndrome and which artery is affected?

Answer 484

Basilar artery (runs right up the center of the pons) - reticular activating system (RAS) is spared therefore consciousness is preserved - quadriplegia - loss of horizontal, but not vertical eye movments

Question 485

How dos the neurodegeneration in vascular dementia differ from that in AD?

Answer 485

Vascular dementia involves a step-wise decline in cognitive ability, with lat-onset memory impairment whereas AD has early recent/short-term memory loss.

Question 486

Which neurodegenerative disease features increased 14-3-3 protein in the CSF?

Answer 486

Creutfeldt-Jakob disease

Question 487

What causes communicating hydrocephalus?

Answer 487

Decreased CSF absorption by arachnoid granulations

Question 488

What causes normal pressure hydrocephalus?

Answer 488

Idiopathic dilation of the ventricles that distorts the corona radiata --> wet (urinary incontinence), wacky (cognitive dysfunction) and wobbly (ataxia)

Question 489

What is a noncommunicating hydrocephalus?

Answer 489

One that's due to blocakge/obstruction

Question 490

What is Lhermitte's phenomenon?

Answer 490

Flexion of the neck in a patient with MS causes them to feel an electric-shock sensation down their spine

Question 491

Increased myelin basic protein in the CSF can be indicative of?

Answer 491

MS

Question 492

What are some of the main features of GBM?

Answer 492

Glioblastoma multiforme (Grade IV astrocytoma) - Most common primary adult tumor - Can cross midline (butterfly glioma) - Has the worse prognosis - GFAP + (makes sense because they are derived from astrocytes) - Can involve both necrosis and hemorrhage - "Pseudopalisading" on histology

Question 493

What are some of the main features of meningioma?

Answer 493

- Often appear in the falx, or parasagital regions - Can fature dural tail - PSaMMoma bodies on histology

Question 494

What are some of the main features of hemangioblastomas?

Answer 494

- HEMANGIO=blood --> can produce EPO and causes 2º polycythmia - Arise from blood vessel (hemangio) - Often occur in the cerebellum - Associated with von Hippel-Lindau syndrome

Question 495

What are some of the main features of schwannomas?

Answer 495

- classically occur at the cerebellopontine angle - often localized to CN VIII - S-100 (+) - "S" for schwannoma

Question 496

What are some of the main features of oligodendrogliomas?

Answer 496

- Rare; slow growing tumors | - "Fried egg" "O's" on histology

Question 497

What is the most common benign childhood brain tumor?

Answer 497

Pilocytic astrocytoma

Question 498

What is the most common malignant childhood brain tumor?

Answer 498

Medulloblastoma

Question 499

What are some of the main features of pilocytic astrocytoma?

Answer 499

- Most common benign childhood brain tumor - Most often found in posterior fossa (e.g. cerebellum) - Glial cell origin - GFAP (+) - May feature Rosenthal fibers and hair like (pilocytic) projections

Question 500

What are some of the main features of medulloblastomas?

Answer 500

- Most common malignant childhood brain tumor - Can compress the 4th ventricle and cause non-communicating hydrocephalus - Features Homer-Wright rosettes = small blue cells surrounding a central area of eosinophilia

Question 501

What are some of the main features of ependymoma?

Answer 501

- Poor prognosis because they can obstruct the 4th ventricle and cause hydrocephalus - features perivascular rosettes (e.g. looks like Homer-Wright rosettes, but instead surrounding a blood vessel)

Question 502

How do craniopharyngiomas differ from pituitary adenomas?

Answer 502

Both lie in the sella turcica, however craniopharyngiomas generally involved HYPOsecretion.

Question 503

What are some of the findings of an uncal herniation?

Answer 503

An uncal herniation occurs when part of the medial temporal lobe slides out and under the tentorium cerebelli causing: - ipsilateral CN III damage - ipsilateral PCA damage (contraltral homonymous hemaniopia with macular sparing) - ipsilateral pareesis

Question 504

Which MN lesion presents with clasp-knife spasticity?

Answer 504

UMN

Question 505

Which MN lesion presents with weakness?

Answer 505

Both UMN and LMN

Question 506

What are the findings/lesions seen in poliomyelitis and Werdnig-Hoffmann disease?

Answer 506

- Both involve the anterior horns of the spinal cord --> cause LMN signs - Werdnig-Hoffmann disease is an autosomal recessive inherited degeneration of anterior horn --> threfore symmetric

Question 507

What are the findings/lesions in amyotrophic lateral sclerosis?

Answer 507

- Combined UMN and LMN deficits with no sensory/bowel/bladder deficits - Can be caused by a defect in superoxide dismutase --> increased free radical damage Treatment: Riluzole (Ri"Lou"zole)

Question 508

What are the findings/lesions in tabes dorsalis?

Answer 508

- Caused by 3º syphilis - Results from degeneration of dorsal columns and roots lading to sensory ataxia - Present with Romberg sign - Treatment: penicillin G

Question 509

What are the findings/lesions in syrngomyelia?

Answer 509

- Syrinx expands and damages the anterior white commissure of the spinothalamic tract --> bilateral loss of P/T sensation - Associated with Chiari I malformation

Question 510

What are the findings of Brown-Séquard syndrome?

Answer 510

Brown-Séquard is a hemisection of the spinal cord that results in: 1. Ipsilateral LMN signs at the level of the lesion 2. Ipsilateral UMN signs below the level of the lesion 3. Ipsilateral loss of all sensation at the level of the lesion 4. Ipsilateral loss of fine touch/proprioception/vibration below level of lesion 5. Contralateral loss of pain/temperature below level of lesion

Question 511

Damage in what nerve causes an afferent pupillary defect?

Answer 511

Optic nerve of the affected eye --> light shown in the affected eye produces a decrease (or dilation) in the pupillary reflex compared to the unaffected eye

Question 512

What type of nephritic syndrome is Goodpasture syndrome?

Answer 512

Rapidly progressive (crescentic) glomerulonephritis

Question 513

What is the difference between basophilic stippling and ring sideroblasts?

Answer 513

Both can be indicative of iron excess or lead poisoning, but stiPPling is seen on PeriPheral smear, while sideroBlasts are seen in Bone marrow.

Question 514

Give an example of HMG-CoA reductase inhibitors. What are their MOA, ADRs and effect on LDL, HDL, and TG.

Answer 514

"statins" - MOA: inhibit conversion of HMG-CoA to mevalonate (cholesterol precursor) - ADRs: hpatotoxic, myopathy (sp. with fibrates or niacin) - LDL: ↓↓↓ - HDL: ↑ - TGs: ↓

Question 515

Give an example of bile acid resins. What are their MOA, ADRs and effect on LDL, HDL, and TG.

Answer 515

"chol" drugs: cholestyramine, colestipol, colesevelam - MOA prevent intestinal reabsorption of bile acids; liver must use cholesterol to make more - ADRs: GI upset - LDL: ↓↓ - HDL: slightly ↑ - TGs: slightly ↑ (think...because they cause increased cholesterol synthesis)

Question 516

Describe ezetimibe's MOA, ADRs and effect on LDL, HDL, and TG.

Answer 516

- MOA: provent cholesterol absorption at small intestine brush border - ADRs: rare increase in LFTs, diarrhea - LDL: ↓↓ - HDL: ↑/— - TGs: ↓/—

Question 517

Give an example of fibrates. What are their MOA, ADRs and effect on LDL, HDL, and TG.

Answer 517

gemfibrozil, bezafibrate, fenofibrate - MOA: upregulate LPL increasing TG clearance (fiBRates BReak up TG) - ADRs: myopathy, cholesterol gallstones - LDL: ↓ - HDL: ↑ - TGs: ↓↓↓

Question 518

Describe niacin's MOA, ADRs and effect on LDL, HDL, and TG.

Answer 518

- MOA: inhibits lipolysis in adipose tissue; reduces hepatic VLDL synthesis - ADRs: red, flushed face, hyperglycemia, hyperuricemia - LDL: ↓↓ - HDL: ↑↑ - TGs: ↓

Question 519

Give an example of PCSK9 inhibitors. What are their MOA, ADRs and effect on LDL, HDL, and TG.

Answer 519

"-cumabs" - alirocumb, evolocumab - MOA: prevent of LDL-receptor degradation --> increase amount of LDL removed from the bloodstream - ADRs: myalgias, delirium/demntia - LDL: ↓↓↓ - HDL: ↑ - TGs: ↓

Question 520

Why should you not administer epinephrine to a patient with acute closed-angle glaucoma?

Answer 520

Epinephrine will cause mydriasis, which will significantly worsen the angle! Do NOT give epinephrine!!

Question 521

Black bone spicule-shaped deposits on the macula and painless progressive vision loss beginning with night blindness should clue you in to which ophthamalogic condition?

Answer 521

Retinitis pigmentosa - an inherited retinal degeneration

Question 522

How does the presentation and cause of CNIII differ for the motor arm vs. parasympathetic arm?

Answer 522

Motor: will present with a down/out gaze - causes are usually related to ischemia and lack of nutrients, since the O2 has to diffuse across the muscle fibers, and can be compromised in cases of vascular disease (e.g. DM) Parasympathetic: will present with a blown pupil and often down/out gaze - usually caused by compression (e.g. increasd IOP, PCom aneurysm, uncal herniation)

Question 523

What drug is used to treat narcolepsy and//or cataplexy?

Answer 523

Modafinil, an atypical dopamine reuptake blocker, is used to treat narcolepsy, although its exact mechanism of action is unknown. Patients suffering from cataplexy may also be treated with sodium oxybate.

Question 524

What is the treatment for serotonin syndrome?

Answer 524

cyproheptadine (5-HT2receptor antagonist

Question 525

In HSV1, which motor protein is responsible for anterograde transport in which is responsible for retrograde transport?

Answer 525

DRAKe: - Dynein = retrograde = latency - Kynesin = anterograde = reinfection

Question 526

How does demyelination affect the time constant and the space constant?

Answer 526

- Demyelination increases the time constant (i.e. causes a decrease in velocity) and decreases the length/space constant (i.e. it decreases the length the electrical impulse can propagate without requiring active regeneration by ion channels)

Question 527

Describe the microscopic changes that occur over time following in ischemic stroke:

Answer 527

- 12–24 hours: read neurons (eosinophilic cytoplasm with pyknotic nuclei) - 24–72 hours: necrosis and neutrophils - 3–5 days: macrophages - 1-2 weeks: reactive gliosis and vascular proliferation

Question 528

What is the most common cause of spontaneous lobar hemorrhage?

Answer 528

Cerebral amyloid angiopathy (particularly in the elderly)

Question 529

What accounts for the rapid onset and relatively short duration of propofol and other highly lipophilic drugs?

Answer 529

The quick onset and fast offset is due to the fact that highly lipophilic drugs first accumulate quickly into the brain, heart and other organs with high blood flow, before being re-distributed (e.g. within 15 minutes) to other tissues such as the skeletal muscle and eventually bone and adipose tissue.

Question 530

What are the most common causes of meningitis in newborns (0 to 6 months)?

Answer 530

- GBS - E. coli - Listeria

Question 531

What are the most common causes of meningitis in patients over 60yo?

Answer 531

- S pneumoniae - Gram (-) rods - Listeria

Question 532

The patient presents with contralateral paralysis in sensory loss, without any cortical signs (e.g. neglect, a facia, visual field loss). What is the artery, brain area most likely affected and what is this usually secondary to?

Answer 532

This is most likely a stroke in the lenticular-striate artery causing a loss of function in the striatum and internal capsule. This is a common location of lacunar infarcts, due to hyaline arteriosclerosis secondary to unmanaged hypertension

Question 533

How do PPV and NPV vary with changes in disease prevalence?

Answer 533

PPV varies directly with pretest probability (i.e. prevalence), while NPV varies inversely with prevalence. Therefore, for a disease with a high prevalence, the test will have a high PPV and a low NPV (therefore, if there are more people with the disease in the population, it is more likely that a positive test result is truly positive, and less likely that a negative test result is truly negative)

Question 534

What are the most common causes of meningitis in children and adults (up to 60yo)?

Answer 534

- S pneumoniae - N meningitidis (#1 in teens) - Enteroviruses - HSV

Question 535

How do PPV and NPV vary with changes in disease prevalence?

Answer 535

PPV varies directly with pretest probability (i.e. prevalence), while NPV varies inversely with prevalence. Therefore, for a disease with a high prevalence, the test will have a high PPV and a low NPV (therefore, if there are more people with the disease in the population, it is more likely that a positive test result is truly positive, and less likely that a negative test result is truly negative)

Question 536

What is the equation for NNH

Answer 536

NNH = 1 / AR | Where AR = attributable risk

Question 537

What is the equation for NNT?

Answer 537

NNT = 1 / ARR | Where ARR = absolute risk reduction

Question 538

What is the equation for NNH

Answer 538

NNH = 1 / AR | Where AR = attributable risk

Question 539

Which embryologic tissues are dervied from surface ectoderm?

Answer 539

- Tissues that are somehow connected to the external environment: MO'S APPLE - Mammary glands - Olfactory epithelium - Adenohypohphysis - Parotid gland - Pectinate line (anal canal below) - Lens of eye - Epidermis

Question 540

Which embryologic tissues are derived from the neural tube?

Answer 540

the Brain and spinal cord (CNS) make up the neural tube - Brain, spinal cord and retina: - Brain (neurohypophysis, CNS neurons, oligodendrocytes, astrocytes, ependymal cells, pineal gland), retina, spinal cord

Question 541

Which embryologic tissues are derived from the neural crest cells?

Answer 541

PNS and non-neural structures nearby: MOTEL BED PASS - Melanocytes - Odontoblasts (teeth) - Tracheal cartilage - Enterochromaffin cells - Laryngeal cartilage - Bones of the skull - Endocardial cushions - Dorsal root ganglia - Parafollicular cells - Adrenal medulla - Schwann cells - Spiral membrane

Question 542

Which embryologic tissues are derived from mesoderm?

Answer 542

Middle = "meat" layer: - Muscle, bone, connective tissue, serous linings of body cavities, spleen, CV structures, lymphatics, blood, wall of gut tube, upper vagina, kidneys, adrenal cortex, dermis, testes, ovaries

Question 543

Which embryologic tissues are derived from endoderm?

Answer 543

"Enternal" layer: - Gut tube epithelium, including anal canal above pectinate line, most of urethra and lower vagina, lungs, liver, gallbladder, pancreas, eustachian tube, thymus, parathyroid, thyroid follicular cells

Question 544

What is the #1 cause of intellectual disability and congenital defects in the US?

Answer 544

Ftal alcohol syndrome

Question 545

How does alcohol use during pregnancy lead to FAS?

Answer 545

Alcohol prevents neural crest cells from migrating

Question 546

What is the most common type of monozygotic twin pregnancy?

Answer 546

Monochorionic, diamniotic (75% of monozygotic twins)

Question 547

Which structures are derived from the 1st-4th branchial pouch?

Answer 547

Ears, tonsils, bottom to top - 1st pouch = ears: middle ear, eustachian tube, mastoid air cells - 2nd pouch = palatine tonsils - 3rd pouch= inferior parathyroids, thymus - 4th pouch - 4th pouch structures migrate above 3rd pouch: superior parathyroids

Question 548

How does a bicornuate uterus differ from uterus didelphys?

Answer 548

Bicornuate uterus is due to a incomplete fusion of Müllerian ducts, results in two uteri with a single cervix. Uterus didelphys is the result of a complete failure of fusion, resulting in a double uterus, cervix and vagina. pregnancy is still possible

Question 549

How do the embryologic causes of hypospadias and epispadias differ?

Answer 549

Hypospadias = abnormal opening of penile urethra on ventral (underside) of penis due to failure of urethral folds to fuse Epispadias = abnormal opening of penile urethra on the dorsal (topside) of penis due to faulty positioning of genital tubercle

Question 550

Which lymph nodes do the head and neck drain into?

Answer 550

Cervical

Question 551

Which lymph nodes do the lungs drains into?

Answer 551

Hilar

Question 552

The mediastinal lymph nodes drain what oragns?

Answer 552

Trachea and esophagus

Question 553

The axillary lymph nodes drain what part of the body?

Answer 553

Upper limb, breast, skin above umbilicus

Question 554

Which structures are drained by the celiac lymph node cluster?

Answer 554

Liver, stomach, spleen, pancreas, upper duodenum (i.e. structures that receive blood supply from the celiac a.)

Question 555

Which structures are drained by the superior mesenteric lymph node cluster?

Answer 555

Lower duodenum, jejunum, ileum, colon to splenic flexure

Question 556

Which structures are drained by the inferior mesenteric lymph node cluster?

Answer 556

Colon from splenic flexure to upper rectum

Question 557

Which lymph node cluster drains the body of the uterus, cervix and superior bladder?

Answer 557

External iliac nodes

Question 558

Which lymph node cluster drains the prostate, cervix, corpus cavernosum, proximal vagina, and lower rectum above the pectinate line?

Answer 558

Internal iliac nodes

Question 559

Which lymph node cluster drains the distal vagina, vulva, scrotum and distal anus?

Answer 559

Superficial inguinal nodes

Question 560

Which lymph node cluster drains the glans penis?

Answer 560

DEEP inguinal nodes

Question 561

Which structures lie within the infundibulopelvic ligament and must therefore be ligated prior to oophorectomy?

Answer 561

Infundibulopelvic ligament = the suspensory ligament of the ovary; contains the ovarian vessels

Question 562

Which structures lie within the cardinal ligament and what other structure is at risk of being cut during hystrectomy?

Answer 562

The uterine vessels lie within the cardinal ligament. The ureter is at risk of injury during ligation.

Question 563

What is the pathway of sperm during ejaculation?

Answer 563

SEVEN UP: - Seminiferous tubules - Epididymis - Vas deferens - Ejaculatory ducts - (Nothing) - Urethra - Penis

Question 564

When can beta-hCG be detected in the blood and in the urine?

Answer 564

Blood: 7 days after fertilization Urine: 14 days after fertilization (i.e. think of a woman doing a pregnancy test after she misses her period - which would have occurs 14 days after ovulation/fertilization)

Question 565

What are the components of the APGAR score?

Answer 565

APGAR: - Appearance: Pink-2pts, Extremities blue-1pts, Everything blue - 0pts - Pulse: > 100bp-2pts, <100bpm-1pt, No pulse-0pts - Grimace: Cries/pulls away-2pts, Grimaces/weak cry-1pt, No response to stimulation-0pts - Activity: Active movement-2pts, Arms and legs flexed-1pt, No movement-0pts - Respiration: Strong cry-2pts, Slow/irregular-1pt, No breathing-0pts

Question 566

What causes Kallmann syndrome?

Answer 566

Failure of migration of GnRH-releasing neurons and subsequent failure of GnRH-releasing olfactory bulbs to develop (results in anosmia)

Question 567

What height should the uterus be at 12 weeks gestation and 20 weeks gestation?

Answer 567

12 weeks: pubic symphisis | 20 weeks: umbilicus

Question 568

Which malignancy of trophoblastic tissue is associated with elevated beta-hCG and cannonball metastases to the lungs?

Answer 568

Choriocarcinoma - suspect in a woman with a history of molar pregnancy and histology shows absence of chorionic villi

Question 569

Exposure to DES in utero causes what vaginal tumor?

Answer 569

Clear cell adenocarcinoma

Question 570

What is sarcoma botryoides?

Answer 570

An embryonal rhabdomyosarcoma variant that affects girls <4yo "botryoides = bunch of grapes"

Question 571

Which proteins do HPV 16 and 18 produce and what do they inhibit?

Answer 571

Produce E6 which inhibits p53 and E7 which inhibits RB

Question 572

Which histologic finding is pathognomonic of HPV infection?

Answer 572

Koilocytes - dense, irregular cytoplasm and perinuclear halo

Question 573

What is the primary insult that leads to PCOS?

Answer 573

Hyperinsulinemia/insulin resistance leads to disregulation of the HPG axis by causing an increase in pulsatile GnRH secretion which increase LH and FSH secretion in a 3:1 ratio

Question 574

Benign ovarian neoplasm with fallopian tube-lik pithlium?

Answer 574

Serous cystadenoma

Question 575

Mucus-secreting lined ovarian neoplam?

Answer 575

Mucinous adenoma

Question 576

Chocolate cyst

Answer 576

Endometrioma - presents with pelvic pain, dysmenorrhea, and dyspareunia.

Question 577

Pale yellow-tan tumor that looks like the bladder?

Answer 577

Brenner tumor (B = bladder)

Question 578

Ovarian tumor that causes a "pulling" sensation in the groin?

Answer 578

Fibroma

Question 579

Germ cell tumor that often present with hyperthyroidism and is the most common ovarian tumor in females 10-30 years old

Answer 579

Mature cystic teratoma (aka dermoid cyst)

Question 580

Which malignant ovarian neoplasm has the pathognomonic finding of "Call-Exner Bodies" and what are they?

Answer 580

Call-Exner Bodies = Granulosa cell tumor; "don't forget to CALL your GRANny"

Question 581

Most common malignant ovarian neoplasm that presents with PSaMMoma bodies?

Answer 581

Serous cystadenocarcinoma

Question 582

Aggressive malignant ovarian neoplasm usually diagnosed before the age 20?

Answer 582

Immature teratoma

Question 583

Common ovarian tumor in adolescents described as having a "fried egg" appearance on histology.

Answer 583

Dysgerminoma

Question 584

Aggressive tumor that is most common in male infants and features "Schiller-Duval bodies" which appear like glomeruli?

Answer 584

Yolk sac tumor

Question 585

GI malignant that metastasizes to ovaries and features "signet cells"

Answer 585

Krukenberg tumor

Question 586

Dilitation and curettage is often associated with which endometrial condition?

Answer 586

Asherman syndrome --> adhesions/fibrosis of the endometrium

Question 587

Which endometrial condition is the most common tumor in females, with a peak in those aged 20-40? This tumor increases in size with pregnancy, decreases with menopause, and is often classified on histology as having a "whorled pattern of smooth muscle"

Answer 587

Leiomyoma

Question 588

A woman in her 50s/60s with vaginal bleeding, should have what DDx considered?

Answer 588

Endometrial carcinoma

Question 589

A collection of serous fluid between the parietal and visceral layers of the tunica vaginalis is known as a:

Answer 589

Hydrocele

Question 590

What is the most common testicular germ cell tumor, and which serum marker is diagnostic?

Answer 590

Seminioma; ALP

Question 591

Prostatic adenocarcinoma is most common in:

Answer 591

Posterior lobe (Peripheral zone)

Question 592

What are the rotator cuff muscles?

Answer 592

SItS - Supraspinatus (abducts arm until deltoid takes over) - Infraspinatus (laterally rotates arm) - teres minor - Subscapularis

Question 593

What are the wrist bones?

Answer 593

From radial to ulnar: "So Long To Pinky, Here Comes The Thumb" - Scaphoid - Lunate - Triquetrum - Pisiform - Hamate - Capitate - Trapezoid - Trapezium

Question 594

Which nerve is commonly injury due to an anterior dislocation of the humerus or fractured cervical neck, and how does this injury present?

Answer 594

- Axillary nerve | - Presents with a flatten deltoid muscle and loss of abduction greater than 15°

Question 595

Which nerve is commonly injury due to compression of the upper trunk, and how does this injury present?

Answer 595

- Musculocutaneous nerve (C5-C7) | - presents with loss of forearm flexion and supination

Question 596

Which nerve is commonly injury due to midshaft fracture of the humerus or compression of the axilla, and how does this injury present?

Answer 596

Radial (C5-T1) | - presents with a wrist drop

Question 597

Traction or tear of the upper trunk (C5-C6) leads to which palsy?

Answer 597

Erb’s Palsy involves the ERBer trunk

Question 598

Traction or tear of the upper trunk (C8-.T1) leads to which palsy?

Answer 598

Total claw hand with loss of fine finger movements

Question 599

An injury to which nerve and subsequently which muscle results in a “winged scapula”?

Answer 599

Long thoracic nerve and serratus anterior muscle

Question 600

A trendelenburg sign/gait is due to a lesion in which nerve/muscles? How can you tell which side is affected?

Answer 600

- Due to an injury of the superior gluteal nerve (L4-S1) which causes weakness in the gluteus medius and gluteus minimus muscles. This injury can occur when there is an intramuscular injection to the upper-medial gluteal region - The lesion is contralateral to the side of the hip that drops (i.e. ipsilateral to the extremity on which the patient stands)

Question 601

Which muscles and which nerve is responsible for hip adduction?

Answer 601

Muscles: adductor Magnus, adductor longus, adductor brevis Nerve: obturator

Question 602

Which muscles and nerves are responsible for hip extension?

Answer 602

- gluteus Maximus (Inferior gluteal nerve) - Semitendinosus - Semimembranousus

Question 603

Which muscles and nerves are responsible for hip flexion?

Answer 603

- Iliopsoas - Rectus femoris - Tensor fascia lata - Pectineus

Question 604

Which group of muscles in which nerve is responsible for internal rotation of the hip?

Answer 604

- nerve: superior gluteal nerve | - muscles: gluteus medius, gluteus minimums, tensor fascia lata

Question 605

Which group of muscles is responsible for external rotation of the hip?

Answer 605

- iliopsoas, gluteus Maximus, piriformis, obturator

Question 606

How does osteoporosis differ from osteopenia?

Answer 606

Osteoporosis: T-score ≤ -2.5 Osteopenia: T-score between -2.5 and -1

Question 607

What would the Ca2+, PO4^3-, ALP, and PTH levels be in a patient with osteoporosis?

Answer 607

All normal - osteoporosis is caused by a loss of mass in trabecular and cortical bone; bone mineralization is still normal and lab values are normal - diagnosis is based on DEXA scan/T-score

Question 608

What would the Ca2+, PO4^3-, ALP, and PTH levels be in a patient with osteopetrosis?

Answer 608

All normal - although sometimes calcium may be low due to decreased osteoclast activity and hence decreased bone resorption

Question 609

What would the Ca2+, PO4^3-, ALP, and PTH levels be in a patient with Paget disease of bone?

Answer 609

Only increased ALP since Paget's is primarily due to a balanced increase in both osteoclastic and osteoblastic activity. Presents with a mosaic pattern of woven and lamellar bone. Hx: increasing hat size with hearing loss (due to narrowing of auditory foramen)

Question 610

What would the Ca2+, PO4^3-, ALP, and PTH levels be in a patient with osteomalacia/rickets?

Answer 610

Calcium: decreased, phosphate: decreased, ALP: increased, PTH: increased. The primary insult here is a deficiency in vitamin D resulting in a defective mineralization of osteoid in osteomalacia and defective cartilaginous growth plates in rickets.

Question 611

Which part of the bone and which age group is generally affected by giant cell bone tumors?

Answer 611

- 20-40 years old | - Epiphysis of long bones

Question 612

Which part of the bone and which demographic is most commonly affected by osteochondroma?

Answer 612

- Metaphysis of bones | - Males < 25yo

Question 613

Which tumor is commonly described as having a sunburst appearance on X-ray? Which part of the bone is affected, what are risk factors, and what is the most common demographic?

Answer 613

Osteosarcoma: - Bimodal: 10-20yo, and >65yo - Most commonly affects the metaphysis Risk factors: Paget disease, familial RB, Li-Fraumeni syndrome

Question 614

Which aggressive bone tumor is described as "small blue cell" with and "onion skin" periosteal reaction in bone? Which part of the bone, which demographic is affected and what is the translocated associated with this tumor?

Answer 614

Ewing Sarcoma - Affects boys < 15yo - Occurs in diaphysis of long bones - t(11;22) translocation

Question 615

What drugs can be used to treat gout acutly?

Answer 615

Focus on pain relief: NSAIDS, glucocorticoids, colchicine

Question 616

What drugs can be used for the chornic treatment of gout?

Answer 616

Preventative: allopurinol (xanthine oxidase inhibitor)

Question 617

Which type/shape of crystals are seen in "pseudogout" and what conditions are commonly associated?

Answer 617

Pseudogout = calcium pyrophosphate deposition disease - Features rhomboid-shaped positively birefringent crystals - Associated with: hemochromatosis, hyperparathyroidism

Question 618

Which MHC molecule is associated with the seronegative spondyloarthropathies, and what are the names of those arthropathies?

Answer 618

HLA-B27: PAIR - Psoriatic arthritis - Ankylosing spondylitis - Inflammatory bowel disease - Reactive arthritis

Question 619

Uvitis and aortic regurgitation is commonly associated with which seronegative spondyloarthropathy?

Answer 619

Ankylosing spondylitis

Question 620

Which antibodies are associated with SLE and what are their respective sensitivities/specificities?

Answer 620

- Antinuclear antibodies (ANA) - sensitive, not specfic - Anti-dsDNA - specific (poor prognosis) - Anti-Smith - specific, but not prognostic - Anti-histone - sensitive for drug-induced lupus (e.g. INH, procainamide)

Question 621

Which antibody is specific and pathognomonic for mixed connective tissue disease?

Answer 621

anti-U1 RNP antibodies

Question 622

A woman > 50yo presenting with pain and stiffness in her shoulders and hips without weakness, most likely has what condition? What other disease is her condition associated with?

Answer 622

She likely has polymyalgia rheumatica, which is often associated with giant cell (temporal) arteritis.

Question 623

How do the antibodies seen in diffuse scleroderma and CREST syndrome (limited) differ?

Answer 623

- Scleroderma: anti-Scl70 (an anti-topoisomerase I Ab) | - CREST syndrome: anti-centromere Ab.

Question 624

Which virus causes molluscum contagiosum?

Answer 624

Poxvirus

Question 625

What is the most common skin cancer?

Answer 625

Basal cell carcinoma

Question 626

Which skin cancer is associated with actinic keratoses and exposure to sunlight?

Answer 626

Squamous cell carcinoma

Question 627

Which skin cancer is associated with rolled/heaped up borders and has palisading nuclei on histology?

Answer 627

Basal cell carcinoma

Question 628

Which skin cancer has the greatest risk for metastasis?

Answer 628

Melanoma

Question 629

Which COX-2 inhibitor is contraindicated in patients with sulfa allergy?

Answer 629

Celecoxib

Question 630

Pencil-in-cup deformity is most commonly seen in what condition?

Answer 630

Psoriatic arthritis

Question 631

What is the most specific antibody for RA?

Answer 631

Anti-citrullinated protein (anti-CCP)

Question 632

What are the lab values for the first-trimester and second-trimester screens for Trisomy 21?

Answer 632

1st trimester: ↓PAPP-A, ↑hCG | 2nd trimester: ↓AFP, ↓estriol, ↑hCG, ↑ inhibin A

Question 633

What are the lab values for the first-trimester and second-trimester screens for Edwards syndrome (trisomy 18)?

Answer 633

1st: ↓PAPP-A and hCG 2nd: ↓AFP, hCG, estriol and inhibin A

Question 634

What are the lab values for the first-trimester screen for Patau syndrome (trisomy 13)?

Answer 634

↓AFP, hCG

Question 635

What is Meigs syndrome?

Answer 635

Triad of: ovarian fibroma, ascites, hydrothorax. “Pulling” sensation in groin.

Question 636

What does the bluish color of the Wright-Giemsa stain of reticulocytes indicate?

Answer 636

Residual rRNA

Question 637

Where does fetal erythropoiesis occur at various stages of development?

Answer 637

“Young Liver Synthesizes Blood” - Yolk sac (3-8 weeks) - Liver (6 weeks-birth) - Spleen (10-28 weeks) - Bone marrow (18 weeks-adulthood)

Question 638

A deficiency in GpIb results in what bleeding disorder?

Answer 638

Bernard-Soulier syndrome

Question 639

A deficiency in GpIIb/IIIa results in what bleeding disorder?

Answer 639

Glanzmann thrombasthenia

Question 640

What pathology is associated with acanthocytes?

Answer 640

Liver disease, abetalipoproteinemia

Question 641

What pathology is associated with basophilic stippling?

Answer 641

Lead poisoning

Question 642

What pathology is associated with dacrocytes?

Answer 642

Bone marrow infiltration (e.g. myelofibrosis)

Question 643

What pathology is associated with degmacytes?

Answer 643

Degmacyte = bite cell | G6PD deficiency

Question 644

What pathology is associated with echinocytes?

Answer 644

Echinocyte = burr cell | End-stage renal disease, liver disease, pyruvate kinase deficiency

Question 645

What pathology is associated with elliptocytes?

Answer 645

Hereditary elliptocytosis, usually asymptomatic; caused by mutation in genes encoding RBC membrane proteins (eg, spectrin)

Question 646

What pathology is associated with macro-ovalocytes?

Answer 646

Megaloblastic anemia

Question 647

What pathology is associated with ringed sideroblasts?

Answer 647

Sideroblastic anemia

Question 648

What pathologies are associated with schistocytes?

Answer 648

- Microangiopathic hemolytic anemias (e.g. DIC, TTP/HUS, HELLP syndrome, mechanical hemolysis)

Question 649

What pathologies are associated with target cells?

Answer 649

HALT: - HbC disease - Asplenia - Liver disease - Thalassemia

Question 650

Which two enzymes in heme synthesis are impaired in lead poisoning?

Answer 650

- Ferrochelatase | - ALA dehydratase

Question 651

Which CD markers are present in Hodgkin Lymphoma?

Answer 651

CD15 and CD30

Question 652

Which non-Hodgkin lymphoma is associated with "waxing and waning" lymphadenopathy?

Answer 652

Follicular lymphoma

Question 653

How do multiple myeloma, Waldenström macroglobulinemia and monoclonal gammopathy of undetermined significance (MGUS) all differ from one another?

Answer 653

- Multiple myeloma: M spike composed mostly of IgG along with symptoms of cancer (CRAB - hyperCalcemia, Renal involvement, Anemia, Bone/back pain) - Waldenström macroglobulinemia - M spike composed mostly of IgM, no CRAB symptoms - MGUS: monoclonal expansion of plasma cells < 10% and no CRAB findings

Question 654

Which leukemia has the histologic finding of "smudge cells"?

Answer 654

Chronic lymphocytic leukemia - "crushed little lymphocytes"

Question 655

This leukemia has a dry tap on bone marrow aspiration, and stains for what protein?

Answer 655

Harry cell leukemia - TRAP

Question 656

What is LAP and what is it a measure of?

Answer 656

LAP = leukocyte alkaline phosphatase and it is a measure of the body actively fighting off infection - LAP is low in CML and high in leukemoid reaction

Question 657

A child presents with lytic bone lesions, a skin rash and recurrent URIs, what is the most likely diagnosis and what markers do these cells express?

Answer 657

Langerhans cell histiocytosis: a group of proliferative disorders of dendritic cells - express: S-100 and CD1a - "Berbeck granules" are characteristic

Question 658

Which drug class is given for acute coronary syndrome (STEMI, NSTEMI, unstable angina)?

Answer 658

ADP receptor inhibitors

Question 659

Hydroxyurea is often given to sickle cell patients. What is the effect/MOA?

Answer 659

Hydroxyurea increases HbF

Question 660

What is the most common type of tracheoesophageal anomalhepay?

Answer 660

Esophageal atresia with distal tracheoesophageal fistula (EA with distal TEF)

Question 661

Which GI organ, supplied by the celiac trunk is derived from mesoderm?

Answer 661

The spleen

Question 662

Which three structures are contained in the hepatoduodenal ligament?

Answer 662

Portal triad: proper hepatic artery, portal vein, common bile duct

Question 663

Which structures distinguish the main three sections of the small intestine?

Answer 663

- Duodenum: Brunner's Glands - Jejunum: Plicae circulares - Ileum: Peyer's Patches

Question 664

How do Zones 1, 2 and 3 of the liver differ based on what substances/toxins/organisms they are susceptible to?

Answer 664

Zone 1: closest to the hepatic triad is most susceptible to ingested toxins and viral hepatitis (1st to contact these things) Zone 2: II = yellow fever Zone 3: closest to the central vein and therefore the least deoxygenated blood, making this zone most susceptible to ischemia, and metabolic toxins (i.e. substances that become toxic after being activated by the liver: alcohol, acetaminophen)

Question 665

This salivary gland tumor is the most common type and features both chondromyxoid stroma and epithelium:

Answer 665

Pleomorphic adenoma

Question 666

This salivary gland tumor is associated with smoking and has a papillary cystic composition with germinal centers:

Answer 666

Warthin tumor (papillary cystadenoma lymphomatosum)

Question 667

This salivary gland tumor is the most common malignant type, and consists of mucinous and squamous components:

Answer 667

Mucoepidermoid carcinoma

Question 668

Barrett esophagus is associated with an increased risk for which type of cancer?

Answer 668

Esophageal adenocarcinoma

Question 669

What are a Virchow node, Krukenberg tumor, and Sister Mary Joseph Nodule?

Answer 669

All represent GI cancer metastases: - Virchow node: left supraclavicular node - Krukenberg: bilateral mets to the ovaries - Sister Mary Joseph nodule: periumbilical met

Question 670

H. pylori is more likely to be associated with which type of peptic ulcer?

Answer 670

Duodenal

Question 671

Which type of peptic ulcer is more likely to be malignant?

Answer 671

Gastric

Question 672

Is a Zenker diverticulum a true or false diverticulum? Which structures are involved?

Answer 672

``` A false diverticulum "Elder MIKE has bad breath" - Elderly - Males - Inferior pharyngeal constrictor - Killian triangel (what the outpouch goes through) - Esophageal dysmotility - Halitosis ```

Question 673

What is the purpose of the pertechnetate (99-Tc) study for Meckel diverticulum?

Answer 673

The pertechnetate study is used to identify ectopic gastric mucosa, as in Meckel's diverticulum,; this is important to distinguish it from acute appendicitis, which can present similarly to this condition.

Question 674

A child who fails to pass meconium within 48 hours of birth, should be worked up for what condition?

Answer 674

Hirschsprung disease

Question 675

What is the most common cause of small bowel obstruction in adults?

Answer 675

Adhesions

Question 676

Which two colonic polyps have malignant potential?

Answer 676

- Adenomatous polyps (associated with AAPC and KRAS mutations; villous > tubular because villous = VILLainOUS) - Serrated polyps (BRAF mutations and CpG hypermethylation results in microsatellite instability)

Question 677

Which polyposis syndrome features supernumary teeth?

Answer 677

Gardner syndrome

Question 678

Which polyposis syndrome features both colonic involvement and CNS tumors?

Answer 678

Turcot syndrome ("TURcot = TURban")

Question 679

Peutz-Jeghers syndrome and Juvenile Polyposis syndrome are characterized by which type of polyps?

Answer 679

Hamartomatous polyps

Question 680

Lynch Syndrome classically involves what section of the colon?

Answer 680

Ascending/proximal colon

Question 681

How do right-sided colon cancer and left-sided colon cancer differ?

Answer 681

Right: more likely to bleed and cause iron deficiency anemia Left: more likely to obstruct the entire lesion and cause an "apple core" lesion ; presents with hematochezia

Question 682

What type of mutation is responsible for Lynch Syndrome?

Answer 682

Microsatellite instability

Question 683

What absolute neutrophil count is needed to diagnose spontaneous bactrial peritonitis?

Answer 683

ANC > 250 cells/mm^3

Question 684

What are an elevated AST and ALT indicative of?

Answer 684

Hepatocyte death/necrosis

Question 685

What are an elevated ALP and GGT indicative of?

Answer 685

Biliary obstruction - note: GGT is specific to biliary disease, and therefore will b normal in bone disease where ALP is elevated

Question 686

Which markers indicate liver function?

Answer 686

- Bilirubin ↑ - Albumin (↑ in advanced liver disease) - Prothrombin time (↑ in advanced liver disease) - Platelets (↓ in advanced liver disease/portal HTN)

Question 687

What is the most common cause of hepatocellular carcinoma in the absence of cirrhosis?

Answer 687

Hepatitis B

Question 688

Which liver cancer is associated with exposure to arsenic and vinyl chloride?

Answer 688

Angiosarcoma

Question 689

What is the primary cause of Budd-Chiari syndrome?

Answer 689

Hypercoagulable states: e.g. polycythemia vera, postpartum state, anti-phospholipid syndrome --> causes nutmeg liver

Question 690

What is the "true" pathophysiology of alpha1-antitrypsin "deficiency"?

Answer 690

The name is a bit of a misnomer, as there is not a true deficiency of this protein that inhibits elastic tissue breakdown, but instead a mutation that causes the protein to be misfolded, and hence non-functional. These aggregates can be visualized with a PAS stain

Question 691

Which hereditary hyperbilirubinemia is associated with stress/fasting? What is the pathophysiology of this disease?

Answer 691

Gilbert syndrome = decreased UDP-glucuronosyltransferase conjugation AND impaired bilirubin uptake

Question 692

What is the difference between Type I and Type II Crigler-Najjar syndrome?

Answer 692

Both have to do with UDP-glucuronosyltransferase - Type I = no activity at all; fatal without liver transplant - Type II = some activity; tx with phototherapy

Question 693

What is the difference between Dubin-Johnson syndrome and Rotor syndrome?

Answer 693

Both cause conjugated hyperbilirubinemia due to defective liver excretion - Dubin-Johnson = black liver - Rotor = no black liver

Question 694

What is the Courvoisier sign and what is it most commonly indicative of?

Answer 694

Painless jaundice = pancreatic cancer

Question 695

What is a common cause of osteomyelitis in patients with sickle cell disease?

Answer 695

Salmonella

Question 696

What is the CD55/59 marker indicative of and what disease are these markers absent in?

Answer 696

CD 55 = decay accelerating factor (DAF) - normally protects RBC membrane from complement CD59 = MAC inhibitory protein These two markers are absent in paroxysmal nocturnal hemobloginuria

Question 697

What are the 11 symptomatic fatures of SLE?

Answer 697

RASH OR PAIN - Rash (malar or discoid) - Arthritis (nonerosive) - Serositis - Hematologic disorders (eg, cytopenias) - Oral/nasopharyngeal ulcers - Renal disease - Photosensitivity - Antinuclear antibodies - Immunologic disorder (anti-dsDNA, anti-Sm, antiphospholipid) - Neurologic disorders (eg, seizures, psychosis)

Question 698

Which fetal component of the placenta synthesizes and secretes hCG?

Answer 698

the SYNcytiotrophoblast SYNthesizes hormones and hCG

Question 699

The 1st branchial cleft develops into what ftal structure?

Answer 699

External auditory meatus

Question 700

The 2nd through 4th branchial clefts develop into what fetal structure?

Answer 700

Temporary cervical sinus which are obliterated by proliferation of 2nd arch mesenchyme.

Question 701

What happens if the cervical sinuses persist?

Answer 701

Branchial clef cyst within the lateral neck (anterior to the sternocleidomastoid)

Question 702

Which factor is the primary determinant of whether testes or ovaries will develop from the undifferentiated gonads?

Answer 702

``` Testes = presence of SRY Ovaries = absence of SRY ```

Question 703

What factor inhibits development of the internal female genitalia and which factor promotes development of male internal genitalia?

Answer 703

The development of female internal genitalia is inhibited by MIH secreted by the sertoli cells. The development of male internal genitalia is promoted by the presence of the mesonephric duct and stimulation by testosterone.

Question 704

Describe the peaks of FSH, LH, Estrogen, and progesterone during the various phases of the menstrual cycle

Answer 704

- FSH and LH are low throughout the entirety of the follicular phase and luteal phase, but peak right at ovulation. - Estrogen: there is a small blip during menses as the follicle develops, a large peak at ovulation since E2 is needed to stimulate the proliferation of the endometrium to prepare for the follicle, and then another peak midway through the secretory face (Luteal) during which the corpus luteum produces both estrogen and progesterone. - Progesterone: peaks during the luteal phase

Question 705

Which cell types have GLUT4 transporters?

Answer 705

Insulin-dependent glucose transporter: | Fat, striated muscle

Question 706

Which cell types have GLUT1 transporters?

Answer 706

RBCs, brain, cornea, placenta

Question 707

Which cell types have GLUT2 transporters?

Answer 707

GLUT2 transporters are bidirectional and are found in the liver, kiney, small intestine and beta islet cells

Question 708

Which cell types have GLUT3 transporters?

Answer 708

brain, placenta

Question 709

Which cell types have GLUT5 transporters?

Answer 709

GLUT5 transporters are fructose transporters and are found in spermatocytes and GI tract (think...they require a lot of fructose to function)

Question 710

Which male and female structures are derived from the genital tubercle?

Answer 710

GeT PC: Penis and clitoris | GeT CV: Corpus cavernosum and Vestibular bulbs

Question 711

Which male and female structures are derived from the urogenital sinus?

Answer 711

US BB: Bulbourethral glands, Bartholin Gland | PUSS: Prostate, Skenes gland

Question 712

Which male and female structures are derived from the urogenital folds?

Answer 712

SUFL: Shaft (ventral), labia minora

Question 713

Which male and female structures are derived from the labioscrotal swelling?

Answer 713

Labia majora and scrotum

Question 714

What does the Paramesonephric (Müllerian) duct develop into and what is the male remnant?

Answer 714

Develops into female internal structures—fallopian tubes, uterus, upper portion of vagina (lower portion from urogenital sinus). Male remnant is appendix testis.

Question 715

What does the Mesonephric (Wolffian) duct develop into and what is the female remnant?

Answer 715

Develops into male internal structures (except prostate)—Seminal vesicles, Epididymis, Ejaculatory duct, Ductus deferens (SEED). In females, remnant of mesonephric duct = Gartner duct

Question 716

Which cells in the CNS are responsible for maintaining the extracellular K+ buffer?

Answer 716

Astrocytes

Question 717

How does the microglia of HIV-infected indivduals appear in the CNS?

Answer 717

They fuse to form multinucleated giant cells

Question 718

Which layer of the peripheral nerve is responsible for the blood-nerve Permeability?

Answer 718

the Perineurium (starts with P)

Question 719

What is the nuclear equivalent of Wallerian degeneration of the axon?

Answer 719

Chromatolysis - the reaction of the neuronal cell body to axonal injury characterized by cellular swelling, displacement of the nucleus to the periphery and loss of Nissl substance

Question 720

Which part of the hypothalamus is responsible for cooling and which is reponsible for heating?

Answer 720

Anterior hypothalamus = A/C (cooling - parasympathetic) Posterior hypothalamus = heating (sympathetic)

Question 721

What is thge role of the limbic system and which structures are involved?

Answer 721

The 5 F's: feeding, fleeing, fighting feeling, sex | Papez circuit includes: hippocampus, mammillary bodies, anterior thalamic nuclei, cingulate gyrus

Question 722

How does a transient ischemic attack differ from an ischemic stroke?

Answer 722

A TIA is a brief, reversible episode of focal neurologic dysfunction, and is not a true infarction, therefore it will have a normal MRI. It is usually just due to a brief period of ischemia. It should be managed with low-dose aspirin.

Question 723

Rupture of which blood vessels results in an epidural hematoma?

Answer 723

Rupture of the middle meningeal artery (branch of maxillary) esp. 2º to skull fracture involving the pterion

Question 724

Where do saccular aneurysms tend to ccur? What is the most common site?

Answer 724

Tend to occur at bifurcations in the circle of Willis (weak areas) - Most common site is the junction of the ACom artery and ACA

Question 725

Which protein in Alzheimer disease, ApoE2 or ApoE4 is associated with an increased risk of the sporadic form of AD?

Answer 725

ApoE4

Question 726

How does Lewy body dementia differ from Parkinson disease?

Answer 726

Lew body dementia presents with parkinsonian features, as well as dementia and visual haLEWYcinations

Question 727

What is another name for Sturge-Weber syndrome, that can be used to help remember its presentation. What are some of the hallmarks of SWS?cherr

Answer 727

Struge-Weber syndrome = encephalotrigeminal angiomatosis (i.e. brain findings and vascular findings in a trigeminal distribution) - Sporadic/Stain(por-wine) - Tram track calcifications (on gyri of brain) - Unilateral - Retardation (due to abnormal neural development caused by GNAQ mutations) - Glaucoma (due to increased IOP)/ GNAQ - Epilepsy (mass effect)

Question 728

How does transference differ from countertransference?

Answer 728

``` Transference = (paTienT to doctor) CountertransferenCe = (doCtor to patient) ```

Question 729

How does depersonalization/derealization disorder differ from conversion (functional neurologic symptom) disorder?

Answer 729

Depersonalization/derealization is characterized by THOUGHTS/FEELINGS of estrangement from one's own self, whereas conversion disorder features SYMPTOMS (e.g. sensory or motor such as blindness, paralysis, etc.) that were triggered by some stressful event.

Question 730

How does the time course and features of schizophrenia, brief psychotic disorder, schizophreniform disorder and schizoaffective disorder differ from one another?

Answer 730

- Schizophrenia: > 6months (diagnosis requires at least 1 positive symptom (delusions, hallucinations, disorganized speech, disorganized speech or catatonic behavior) and negative symptoms - Schizophreniform disorder: lasting 1-6months - Brief psychotic disorder: lasting <1 month - Schizoaffective disorder: meets criteria for schizophrenia in addition to major mood disorder; hallucinations/delusions must be present on their own for > 2weeks

Question 731

What is the #1 risk factor for suicide completion?

Answer 731

Previous attempt (increases risk by 5-6x)

Question 732

How dos schizoid personality disorder differ from avoidant personality disorder?

Answer 732

- Schizoid = Cluster A ("weird") - they voluntary withdraw from society and are content with this isolation - Avoidant = Cluster C ("worried") - they are hypersensitive to rejection, and want to have relationships with others but are too cowardly

Question 733

How does somatic symptom disorder differ from illness anxiety disorder?

Answer 733

Somatic symptom disorder = patient has real bodily complaints/symptoms, and worries about them excessively Illness anxiety disorder = hypochondriasis; patient doesn't actually have any symptoms but is worried about acquiring a serious illness/disease

Question 734

Which cytokines induce differentiation of Th1 cells, what is the function of Th1 cells and which cytokines are they inhibited by?

Answer 734

- Induced by: IL-12, IFN-gamma - Inhibited by IL-4 and IL-10 (secreted by Th2 cells) - Function: activate macrophages, cytotoxic T cells, and induce class switching

Question 735

Which cytokines induce differentiation of Th2 cells, what is the function of Th2 cells and which cytokines are they inhibited by?

Answer 735

- Induced by: IL-2, IL-4 - Inhibited by: IFN-gamma (secreted by Th1 cells) - Function: Th2 achoo! --> induce IgE

Question 736

Which cytokines induce differentiation of Treg cells, what is the function of Treg cells?

Answer 736

- Induced by: TGF-beta (also secrete IL-10, TGF-beta) | - Function: help maintain immune tolerance by suppressing CD4 and CD8 T-cell effector functions

Question 737

How does Alzheimer disease differ from vascular dementia?

Answer 737

- Alzheimer disease is the second most common cause of dementia in the elderly. It usually presents with a slow-onset of memory loss (short term goes first) as well as decreased ability to perform activities of daily living (e..g. driving, groceries) - Vascular dementia is the 2nd most common cause of dementia in the elderly. It presents with a step-wise decline in cognitive ability with late-onset memory impairment.

Question 738

Describe which branches of the nervous system are responsible for the three phases of the male sexual response: erection, emission and ejaculation

Answer 738

"Point, Squeeze, and Shoot" - Erection (Point - Parasympathetic via pelvic nerve) - Emission (Squeeze - Sympathetic peristalsis of the semen via hypogastric nerve) - Ejaculation (Shoot - Somatic via pudendal nerve)

Question 739

What is CD55?

Answer 739

CD55 = Decay-accelerating factor - an inhibitor of complement - Deficiencies in DAF/CD55 cause complement-mediated lysis of RBCs and paroxysmal nocturnal hemoglobinuria (DAF normally prevents activation of complement against "self" cells)

Question 740

Which cytokines are secreted by macrophages and what are their functions?

Answer 740

"1 Macrophage 8((ate) 6 Tumors (TNF-alpha)" - IL-1 --> fever - IL-8 --> chemotactic for neutrophils "Clean up on IL-8" - IL-6 --> causes fever and stimulates acute phase proteins - IL-12 --> (macros) induces differentiation of T cells into Th1 cells - TNF-alpha --> activates endothelium, causes WBC recruitment and vascular leak; causes cachexia, IMPORTANT: maintains granulomas in TB

Question 741

Which cytokines are secreted by all T cells and what are their functions?

Answer 741

Hint: Michael Jordan = #2-3 the GOAT- greatest of ALL T-cells - IL-2 --> stimulates growth of helper, cytoxic and regulatory T cells the "T" in HOT T-B stEAK - IL-3 --> supports growth and differentiation of bone marrow stem cells. The "B" in HOT T-B stEAK

Question 742

Which cytokine is secreted by Th1 cells and what is its function?

Answer 742

IFN-gamma --> secreted by NK cells and T cells in response to antigen; stimulates macrophage and inhibits Th2

Question 743

Which cytokines are secreted by Th1 cells and what are their functions?

Answer 743

- IL-4 --> induces differentiation of T cells into Th2 cells; enhances class switching to IgE and IgG "E" in HOT T-B stEAK - IL-5 --> stimulates eosinophils, and enhances class switching to IgA "A" in HOT T-B stEAK - IL-10 --> atTENuates inflammatory response (along with TGF-beta)

Question 744

Which blue-green hme-containing pigmenet gives sputum its color?

Answer 744

Myeloperoxidase

Question 745

How do aplastic anemia, Diamond-Blackfan anemia, and Franconia anemia all differ from one another?

Answer 745

- Diamond-Blackman anemia = a rapid onset anemia caused by an intrinsic defect in erythroid precursor cells, that also presents with craniofacial abnormalities and triphalangeal thumbs - Aplastic anemia = an acquired pancytopenia caused by failure or destruction of myeloid stem cells (e.g. radiation, viruses, idiopathic) - Fanconi anemia = a genetic DNA repair defect that causes bone marrow failure leading to pancytopenia, but also accompanied by short stature, thumb/radial defects, cafe-au-lait spots and increased risk for cancer.

Question 746

What is the relationship of the pulmonary artery to the bronchus at each lung hilum?

Answer 746

RALS: - Right pulmonary artery is Anterior to the hilum - Left pulmonary artery is Superior to the hilum

Question 747

Which kidney structures are derived from the metanephric mesenchyme?

Answer 747

- all tubing from the glomerulus to the DCT (i.e all the stuff in the middle)

Question 748

Which arteries supply the upper, middle and lower 1/3 of the ureter?

Answer 748

R.CG.I - upper = renal artery - middle = common gonadal - lower = internal iliac

Question 749

What are the percentages for the body fluid compartments?

Answer 749

60% of a person’s body mass is made up of water (the other 40% is non-water substance) - composing that total body water 40% of the TBM is ICF (i.e. RBCs) and 20% is ECF (plasma and interstitial fluid) - Plasma makes up 25% of the ECF and interstitial fluid makes up 75%

Question 750

What is the quation for Henderson-Hasselbalch?

Answer 750

pH = 6.1 + log (HCO3/0.03PCO2)

Question 751

What is the Winters formula, what is it used for, and how are the values interpreted?

Answer 751

The Winters formula is used to predict the appropriat/expected respiratory compensation for a simple metabolic acidosis. PCO2 = 1.5[HCO3-] + 8 ± 2 - If measured PCO2 > Predicted = concomitant respiratory acidosis - If measured PCO2 < Predicted = concomitant respiratory alkalosis

Question 752

How does the presentation of acute posstreptococcal glomerulonephritis (PSGN) differ from IgA nephropathy (Berger disease)?

Answer 752

In PSGN, the renal symptoms occur ~2-4 weeks following a Group A strep infection of the pharynx or skin. IgA nephropathy features episodic gross hematuria that occurs concurrently with the respiratory or GI infection.

Question 753

What is the relationship of the pulmonary artery to the bronchus at each lung hilum?

Answer 753

RALS: - Right pulmonary artery is Anterior to the hilum - Left pulmonary artery is Superior to the hilum

Question 754

What is the equation for the "alvolar gas equation"

Answer 754

PAO2 = PIO2 - PaCO2/0.8 - PAO2 = alveolar PO2 - PIO2 = PO2 of inspired air (usu. 150mmHg at sea level) - PaCO2 = arterial PCO2

Question 755

Describe the relationship between P(A), P(a), and P(v) in zones 1, 2 and 3 of the lungs:

Answer 755

Note: in all cases P(a) > P(v) --> the only thing that changes in P(A) from Zone 1 to Zone 3 - Zone 1 (apex/top of lungs --> air rises): P(A) > P(a) > P(v) - Zone 2 (middle of lungs): P(a) > P(A) > P(v) - Zone 3 (bottom/base of lungs --> blood sinks): P(a) > P(v) > P(A)f

Question 756

In performing the McMurray maneuver for a meniscal tear, how do you know if the tear is medial or lateral?

Answer 756

LIME: - Pain on Internal rotation = Lateral meniscus - Pain on External rotation = Medial meniscus

Question 757

Which three ligaments are injured in the "unhappy triad"?

Answer 757

An unhappy triad is a common injury in contact sports in which a lateral force applied to a planted leg damages the: - MCL - ACL - MM

Question 758

Where is specifically in the cell dies steroid synthesis take place?

Answer 758

In the smooth endoplasmic reticulum

Question 759

Which enzyme and which cofactor are implicated in maple syrup urine disease?

Answer 759

- Enzyme: branched-chain alpha-ketoacid dehydrogenase | - Cofactor: thiamine (Vit. B1)

Question 760

What are the findings in homocystinuria?

Answer 760

HOMOCYstinuria: - Increased Homocysteine in urine - Osteoporosis - Marfanoid habitus - Ocular chantes (downward lens subluxation) - Cardiovascular effects - kYphosis

Question 761

Which enzyme is deficient in Von Gierke disease?

Answer 761

- Glucose-6-phosphatase --> unable to breakdown glycogen in liver, resulting in severe fasting hypoglycemia

Question 762

Which enzyme is deficient in Pompe disease?

Answer 762

- lysosomal acid alpha-1,4/1,6-glucosidase (acid maltase)

Question 763

Which enzyme is deficient in Cori disease?

Answer 763

- debranching enzyme (alpha-1,6-glucosidase) - limit dextrin accumulates

Question 764

Which enzyme is deficient in McArdle disease?

Answer 764

- myophosphorylase

Question 765

While most drugs exhibit first-order metabolism, which drugs show zero-order metabolism?

Answer 765

PEA: - Phenytoin - Ethanol (at certain concentrations) - Aspirin

Question 766

What are the percentages for ±1, 2, and 3standard deviations from the mean?

Answer 766

±1 = 68% ±2 = 95% ± 3 = 98.5%

Question 767

Which neoplasm presents with cutaneous lesions, hypercalcemia and is common to Japan and Caribbean, ? Which virus causes this neoplasm?

Answer 767

- Adult T-cell lymphoma --> causd by HTLV

Question 768

What is the mechanism of Type 1 RTA?

Answer 768

- Defect in H+ excretion in the DCT leads to impaired reabsorption of K+ - Urine pH > 5.5 (basic because no acid) and Hypocalcemia (because no K+ reabsorption) - Causes: amphotericin B

Question 769

What is the mechanism of Type 2 RTA?

Answer 769

- Impaired total reabsorption in the PCT results in low HCO3, low K+, and low phosphate (can result in hypophosphatemic rickets) - Causes: Fanconi syndrome

Question 770

What is the mechanism of Type 4 RTA?

Answer 770

- Hypoaldosteronism impairs the ability to reabsorb H+ leading to acidic urine (urine pH < 5.5) and hyperkalemia

Question 771

What is the presentation of serotonin syndrome?

Answer 771

The three “A’s” - hyperActivity (clonus, hyperreflexia, hypertonic, seizures) - Autonomic stimulation (hyperthermia, diaphoresis, diarrhea) - Agitation