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STEP1 - TLe > QB - DISEASES(2) > Flashcards

QB - DISEASES(2) Flashcards

1
Q 
Painless hematuria ("coca cola urine") 2-3 days after URI in child/young adult
Deposition of substance found in mesangium
A

IgA nephropathy (Berger disease)

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2
Q

IgA nephropathy;
purpuric lesions on extensor surfaces (arms, legs, butt);
abdominal pain/vomiting/intestinal bleeding/intussusception

A

Henoch-Schonlein disease

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3
Q

Differences btwn IgA nephropathy and Post-Strep GN

A

IgA nephropathy:
develops few days after infection
IgA mesangial deposition

Post-Strep GN:
develops WEEKS after infection
C3 levels low

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4
Q

VIPomas

A

excess water, Na+, and K+ loss in stool
inhibits gastric acid secretion

watery diarrhea
hypokalemia
achlorhydria

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5
Q

Why are patients w/ Crohn’s more susceptible to gallstones?

A

Terminal ileum reabsorbs bile acids – in Crohn’s, the terminal ileum most often affected –> no reabsorption of bile

Ratio of cholesterol:bile acid increases –> cholesterol precipitates in bile of the gallbladder as gallstones

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6
Q

Retroperitoneal hematoma from MVA - possible organs involved?

A

Abdominal aorta, inferior vena cava (rapid blood loss –> hemorrhagic shock)

Pancreas (except tail), kidneys, adrenal glands
parts 2, 3, and portion of 4 of the duodenum
ascending & descending colon, rectum
ureters, bladder

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7
Q

Intraperitoneal structures that can cause hemoperitoneum

A

transverse colon, liver, spleen

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8
Q

Cardiac defect in Turner’s

A

Bicuspid aortic valve

early systolic, high frequency click heard over cardiac apex
can progress to aortic stenosis/regurg

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9
Q

Cardiac defect in Down syndrome

A

ASD
fixed splitting of second heart sound

VSD
holosystolic murmur

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10
Q

Cardiac defect in rheumatic heart disease

A

mitral stenosis

mid-diastolic, low-pitched rumbling murmur (may begin w/ opening snap)

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11
Q

Cardiac defect in Marfan and Ehlers-Danlos

A

MVP

Mid-systolic click, late systolic murmur

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12
Q

Cardiac defect in premature infants w/ respiratory distress syndrome

A

PDA

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13
Q

Trousseau’s sign; what is it an indication of?

A

Migratory superficial thrombophlebitis – superficial venous thromboses appearing in multiple sites and resolving

Indicates visceral cancer - esp. adenocarcinoma of pancreas, colon, lung

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14
Q

Prinzmetal’s (variant) angina – treatment?

A

Coronary artery vasospam

Nitroglycerin (or other vasodilators)

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15
Q

Acute transmural myocardial infarction (MI)

A

peaked T waves (localized hyperkalemia)
ST segment elevation
Q waves

anterior: V1-V3
I, aVL: lateral

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16
Q

Stable angina

A

Stable atheromatous lesion WITHOUT thrombus

Obstructs greater than 75% of coronary artery lumen

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17
Q

Bronze diabetes

A

Skin hyperpigmentation
Diabetes mellitus
Pigment cirrhosis w/ hepatomegaly

Associated w/ late stage hemochromatosis

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18
Q

Hemochromatosis

A

High iron GI absorption

Hepatomegaly
Skin hyperpigmentation (sun exposed areas)
Diabetes mellitus (secondary to pancreatic islet destruction)
Arthropathy
Cardiac dysfunction & enlargement

Elevated plasma iron, serum ferritin, >50% transferrin saturation

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19
Q

Wilson’s disease

A 
Pt. younger than 30 yrs. old
Unexplained chronic hepatitis
Low serum ceruloplasmin
Increased urinary copper excretion
Kayser-Fleischer rings
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20
Q

Budd-Chiari syndrome

A

Thromboti occlusion of hepatic veins and/or the intra/suprahepatic inferior vena cava

Sinusoidal pressure increae –> portal HTN, ascites, hepatomegaly, splenomegaly

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21
Q

Presystolic sound immediately preceding first heart sound - what condition?

A

S4 gallop is presystolic sound immediately before S1

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22
Q

Best location to hear left vs right sided S4 gallop

A

Left sided S4 - cardiac apex with pt. in left lateral decubitus position

Right sided S4 - lower left sternal border (tricuspid area) w/ patient in supine position

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23
Q

Why does S4 arise?

A

Sudden rise in end diastolic ventricular pressure –> atrial contrac against a stiff ventricle (RVH or LVH)

ventricular hypertrophy decreased ventricular compliance –> diastolic dysfunction

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24
Q

What does S1 and S2 represent?

A 
S1 = closing of AV valves
S2 = closing of semilunar valves (pulmonic and aortic)
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25
Q

Cutaneous melanoma gene mutation

A

Mutated BRAF gene (protein kinase) –> activate signaling pathways –> melanocyte proliferation, metastasis

Substitute a glutamic acid for valine (correct) at position 600

Main risk factor for cutaneous melanoma = UV exposure

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26
Q

Glioblastoma causes overexpression of:

A

growth factors (PDGF, EGFR, IGF, TGF)

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27
Q

Taste & sensory sensation to anterior 2/3 and poster 1/3 of tongue?

A

anterior 2/3:
Sensory (pain) - CNV3 (mandibular branch of trigeminal)
Taste - CNVII (facial)

posterior 1/3:
sensory & taste - CNIX

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28
Q

Hydrocele development - cause?

A

Fluid-filled peritoneal sac within scrotum due to incomplete obliteration of processus vaginalis

processus vaginalis is projection of peritoneal cavity that accompanies descending testis into scrotum

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29
Q

Bright red central papule w/ outwardly radiating vessels that blanch w/ pressure and refill on release; what are they and what are they dependent on?

A

Spider angiomas

Dependent on estrogen

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30
Q

Soft, blue compressible masses - appears on skin, mucosa, deep tissues, viscera; have dilated vascular spaces with thin-walled endothelial cells; based in dermis – found in brain and viscera - associated with what disease?

A

Cavernous hemangiomas - associated w/ von Hippel-Lindau disease

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31
Q

Lymphatic cysts lined by thin endothelium - benign tumors present at birth located on neck and lateral chest wall; what disease associations?

A

Turner syndrome

Down syndrome

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32
Q

Cherry hemangioma vs. Strawberry hemangiomas

A

Cherry - in adults - increase in number w/ age – proliferation of capiallaries and post-capillary venules in papillary dermis

Strawberry - in children – present in first weeks of life - regress spontaneously by 5-8yrs

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33
Q

Treatment for diabetic ketoacidosis

A

IV hydration with normal saline & insulin

Insulin decreases lipolysis, decrease production of ketone bodies (increase plasma bicarbonate), decrease K+, decrease glucose, decrease osmolality

Increases sodium and serum bicarbonate

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34
Q

Symptoms of DKA

A

mental status changes, dehydration, abdominal pain, tachypnea, fruity odor in breath

hyperpnea (Kussmaul respirations) - deep slower breathing to have compensatory respiratory alkalosis (due to metabolic acidosis condition)

increased anion-gap metabolic acidosis

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35
Q

Opioid antagonists uses

A

treats opioid overdoses –> respiratory acidosis due to hypoventilation

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36
Q

Winter’s formula

A

PaCO2 = (1.5 x HCO3-) + 8 +- 2

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37
Q

Winter’s formula (when to use it?)

A

Used to evaluate respiratory compensation when there is metabolic acidosis (diabetic ketoacidosis)

PaCO2 > predicted: concurrent respiratory acidosis

PaCO2 < predicted: concurrent respiratory alkalosis

If PaCO2 persists above range given by Winter’s formula –> patient unable to compensate - has mixed acid-base disturbance consisting of metabolic and respiratory acidoses –> respiratory failure

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38
Q

Elevated AFP

A

Hepatocellular carcinoma (HCC)

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39
Q

Elevated CEA

A

Colorectal cancer

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40
Q

Elevated CA-125

A

Ovarian cancer

other cancers:
endometriosis
lung
breast
pancreatic* cancer
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41
Q

Acid phosphatase elevation

A

Secondary prostate tumor marker antigen

also in patients w/ active osteoclast-initiated bone resorption

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42
Q

hCG

A

suspected gestational trophoblastic disease

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43
Q

Physical manifestations of Marfan’s

A

spinal scoliosis and/or kyphosis
lens dislocation
cystic medial degeneration of aorta –> aortic dissection (cause of death)

defect in glycoprotein fibrillin-1

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44
Q

p53 gene inactivation – 2 hits necessary for malignancy; what is disease w/ inherited mutation in p53 (1 hit already, increasing risk of potentially knocking out both p53)

A

Li-Fraumeni syndrome

several cancers appear
cancers occur at young age
cancers appear several times throughout lifetime

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45
Q

Follicular lymphoma

A

Bcl-2 (proto-oncogene) overexpression –> antiapoptosis of tumor cells (immortality)
t(14;18)

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46
Q

CML

A

Bcr-abl

t(9;22) Philadelphia chromosome

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47
Q

Burkitt’s lymphoma

A

EBV
C-myc overexpression
t(8;14) – heavy chain on chromosome 14
High mitotic index w/ high cell death rate
Benign macrophages engulf cellular debris –> clear spaces around macrophages = “starry sky appearance”
adolescents and young adults

African form: jaw lesion
Endemic form: pelvis or abdomen involved

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48
Q

Patient hospitalized for acute MI dies 3-7 days after onset of MI; why?

A

LV free wall rupture (complication)
Occurs when coagulative necrosis + neutrophil infiltration have weaked infarcted myocardium = wall is damaged and more likely to rupture

symptoms: profound hypotension & shortness of breath; jugular venous pressure elevation

LV hypertrophy and prior MI decreases chance of free wall rupture

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49
Q

Most common cause of in-hospital death due to MI

A

LV failure/cadiogenic shock

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50
Q

Frequent complication of cornary artery fibrinolysis (to get rid of clot)

A

Systemic bleeding, especially intracranial hemorrhage

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51
Q

Joint pain + cardiac murmur

A

rheumatic heart disease

antibodies against M-proteins of Streptoccocci

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52
Q

Nasal ulcer + hematuria

A

Wegeners’s (granulomatosis w/ polyangiitis)

upper resp. tract (sinusitis, nasal ulceration)
lower resp. tract (hemoptysis)
kidneys (RPGN)
c-ANCA
pauci-immune: no anti-GBM antibodies or immune complexes

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53
Q

malar rash + pleural effusion

A

SLE

circulating immune complex nephritis
dsDNA & anti-Sm

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54
Q

dysphagia & sclerodactyly

A

CREST syndrome

calcinosis, Raynaud’s, esophageal dysmotility, scerodactyly, telangiectasia

anti-centromere antibodies

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55
Q

Pt. w/ choking spells, dysphagia, coughing; recurrent pneumonia; what could be cause of choking spells?

A

Cricopharyngeal muscle dysfunction – diminished relaxing of pharyngeal mm. during swallowing –> increased pressure in pharynx eventually causes mucosal to herniate out = Zenker diverticulum (false)

diverticulum palpated as lateral neck mass
food retention in diverticulum –> regurgitation occurs days later –> aspiration pneumonia

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56
Q

Congenital hydrocele

A

Processus vaginalis remains patent –> fluid from peritoneum accumulates in scrotum

normal development: testis descends through inguinal canal; peritoneal tissue also descend = processus vaginalis

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57
Q

Young black woman; malaise, cough, cutaneous findings (including erythema nodosum - painful shin nodules); bilateral hilar lymphadenopathy; non-caseating granuloma

A

Sarcoidosis

elevated ACE levels
chronic restrictive granulomatous interstitial lung disease

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58
Q

Cellular immunity

A

T -cell response (NOT B-cell response)

Vaccines generally stimulate B-cells –> memory B lymphocytes & humor immunity

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59
Q

Wrist drop – radial nerve mononeuropathy; causes?

A

Compression
Trauma
Diabetic vasculopathy
Vasculitis

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60
Q

Antibodies against neutrophil myeloperoxidase

A

p-ANCA (perinuclear staining antineutrophil cytoplasmic antibodies)

microscopic polyangiitis or Churg-Strauss vasculitis

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61
Q

Churg-Strauss

A 
idiopathic systemic vasculitis
adult-onset asthma
eosinophilia
mono or polyneuropathy
migratory/transient pulmonary infiltrates
paranasal sinus abnormalities
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62
Q

Mesothelioma

A

Person works in shipyard - exposure to asbestos
neoplasm of plerua/peritoneum
hemorrhagic pleural effusions & pleural thickening

EM gold standard for diagnosis: numerous long slender microvilli & abundant tonofilaments

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63
Q

Bronchioalveolar carcinoma

A

type of adenocarcinoma
PERIPHERY of lung (peripheral mass) - pneumonia like consolidation
distribution along alveolar septa

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64
Q

Small cell carcinoma

A

Major bronchi
hilar mass
Neuroendocrine markers - chromogranin & synaptophysin+

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65
Q

Squamous cell carcinoma

A

hilar mass
keratinization & intercellular bridges
cavitary lung lesion

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66
Q

Mantle cell lymphoma

A

t(11;14)
Immunoglobulin heavy chain (IgH) is on 14
cyclin D1 – promoter of G1 to S-phase transition

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67
Q

Mutations of DNA repair enzymes

A 
Breast cancer
Ovarian cancer
Lynch syndrome
Ceroderma pigmentosum
Fanconi anemia
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68
Q

Hemophilia A

A

X-linked (affects males)
factor VIII deficiency
prolonged PTT
hemorrhage into joints (hemarthrosis); prolonged bleeding after surfical procedures

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69
Q

Hemophilia B

A

factor IX deficiency
Christmas disease
prolonged PTT

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70
Q

Protein C deficiency

A

Lack of protein C –> procoagulation

Suffer from recurrent deep venous thromboses

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71
Q

Deficiency in factors II, V, VII, X, & fibrinogen; effect seen in PT or PTT?

A

PT prolongation

Warfarin – blocks coagulation factors –> also causes PT prolongation

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72
Q

Mucocutaneous bleeding (recurrent epistaxis, gingival hemorrhage); what type of blood disorder?

A

Defect in platelets –> bleeding time prolongation

also seen in vWF disease and NSAIDs

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73
Q

What does thrombin time measure?

A

rate of conversion of fibrinogen –> fibrin

thrombin time prolonged when there is deficiency/defective fibrinogen

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74
Q

Low haptoglobin is an indication of?

A

Hemolytic anemia

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75
Q

Hirschsprung disease

A

Neural crest cells migrate caudally –> rectum and anus always involved if neural crest cells cease to move in development; sigmoid colon involved 75% of time

Absence of parasympathetic ganglion cells = bowel lumen smaller due to inability to relax

Newborns fail to pass meconium - intestinal obstruction –> bilous vomiting, abdominal distension; tone of anal sphincter usually increased

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76
Q

Pseudogout vs. gout

A

Pseudogout:
Knee joint
Rhomboid-shaped positive birefringent
Calcium pyrophosphate crystals

Gout:
1st metatarsophalangeal joint
Monosodium urate (salt of uric acid)
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77
Q

Calcific tendonitis

A

Calcium hydroxyapatitie crystals in periarticular soft tissue (esp. tendons)

Rotator cuff usually affected

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78
Q

Xanthomas

A

Soft tissue deposits of cholesterol
Hyperlipidemia

Yellow papules on knees, elbows, tendon insertion sites

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79
Q

Reye syndrome

A

Salicylates given to patients 5-14yrs old.

Hepatic dysfunction – vomiting & hepatomegaly
Microvesicular steatosis
Increased levels of ALT, AST, ammonia, bilirubin, prolonged PT and PTT

Encephalopathy - hyperammonemia –> cerebral edema

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80
Q

The only disease in which ASA is administered to children under 16yrs

A

Kawasaki disease

Vasculitis of medium-sized arteries (affects children)

Conjunctivitis
Cervical lymphadenopathy
Periungal desquamation (fingers)
Mucocutaneous changes (strawberry tongue)

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81
Q

Centrilobular congestion

A

Occurs in liver of patients w/ right sided heart failure

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82
Q

Primary biliary cirrhosis

A

Autoimmune destruction of intrahepatic bile ducts; granulomatous inflammation: infiltration of macrophages, lymphocytes, plasma cells, eosinophils

Cholestasis

Middle aged woman w/ PRURITUS (more severe at night), PALE stool, xanthelasma (suggestive of cholestasis)

Hepatosplenomegaly

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83
Q

Male w/ UC presents w/ fatigue, high alkaline phosphatase

A

Primary sclerosing cholangitis

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84
Q

Preload

A

The end volumetric pressure that stretches the right/left ventricle of the heart - initial stretching of cadriomyocytes prior to contraction (related to sarcomere length at end of diastole)

VOLUME in heart after LV filling

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85
Q

Afterload

A

Tension or stress developed in the wall of left ventricle during ejection (load against which heart has to contract to eject blood) – PRESSURE that heart LV has to pump against and overcome to eject blood

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86
Q

Why is the pO2 in the left atrium lower than in the pulmonary capillaries?

A

Bronchial arteries return deoxygenated blood (blood that has traveled to supply bronchi and bronchioles) to the pulmonary veins – this results in venous admixture in the pulmonary veins of deoxygenated blood from bronchial arteries & oxygenated blood from the lungs

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87
Q

Determining acid-base disturbance; normal pCO2; normal HCO3-?

A

pH > 7.45 alkalosis
pH < 7.35 acidosis

pCO2 normal = 40
HCO3- normal = 24

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88
Q

Effect of heroin overdose on blood gases?

A

Heroin suppresses respiratory centers –> hypoventilation w/ rentention of CO2 (acute phase)

In chronic respiratory acidosis - kidneys compensate by excreting H+ and reabsorbing HCO3-

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89
Q

When does contraction alkalosis occur? Lab findings?

A

Overuse of diuretics –> volume loss –> increase in aldosterone –> increase rention of Na+ and H2O –> loss of K+ and H+ in kidneys –> loss of H+ causes metabolic alkalosis (contraction alkalosis) bc H+ is traded for HCO3- reabsorption –>

lab findings: high pH, high HCO3-, high pCO2 (CO2 retained to compensate for alkalosis)

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90
Q

Blood gas findings in diabetic ketoacidosis?

A

low pH, low HCO3- (to neutralize accumulated acidic products) leading to metabolic acidosis, low pCO2 (hyperventilation or Kussmaul breathing to compensate for acidosis)

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91
Q

How does brown fat generate heat?

A

Have several intracytoplasmic fat droplets
Contain more mitochondria than white adipose

Uncouples oxidative phosphorylation with protein thermogenin

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92
Q

Restrictive cardiomyopathy

A

reduced LV compliance – diastolic dysfunction –> CHF
restrictive cardiomyopathies = amyloidosis, sarcoidosis, metastatic cancer, products of inborn metabolic errors

contrast with:
dilated cardiomyopathy – systolic dysfunction
viral myocarditis, alcohol toxicity, diphtheritic myocarditis,

doxorubicin (adrianmycin) & daunorubicin – chemo agents also cause dilated cardiomyopathy

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93
Q

Oral thrush - what types of popn. suffer from this infection?

A 
Denture wearers
Diabetics
Immunosuppressed patients (HIV)
Steroid use
Antibiotics, chemotherapy
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94
Q

Luekoplakia

A

Precancerous lesions w/ white patches that can’t be scraped off

contrast w/ candida infection (oral thrush) – contains white patches that CAN be scraped off

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95
Q

Animal contact – fungal infection

A

Microsporum species causing dermatophytosis

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96
Q

Varicose veins - causes and complications?

A

Cause: incompetent venous valves, venous stasis/congestion, edema, increased thrombosis

Complications: painful thromboses, stasis dermatitis, skin ulcerations, poorly healing wounds, superficial infections
NO pulmonary embolism – this is only seen in DEEP venous thromboses (not superficial!)

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97
Q

Cavernous hemangiomas

A

Soft blue compressible masses

on light microscopy – large dilated vascular spaces

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98
Q

Presence of schistocytes

A 
Microangiopathic hemolytic anemia (TTP, HUS, DIC)
Mechanical damage (prosthetic valve)
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99
Q

HUS vs. TTP; similarities and differences?

A

Similar symptoms: fever, neurologic manifestations, renal failure, thrombocytopenia, microangiopathic hemolytic anemia

TTP: adults - neurological symptoms predominate

HUS: children - renal involvement predominate

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100
Q

K+ sparing diuretics; location of action?

A

Spironolactone
Amiloride
Triamterene

K+ sparing diuretics work at collecting duct

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101
Q

Right-sided endocarditis

A

S. aureus – in IV drug users

Perforations to heart valves, rupture of chordae tendinae, septic emboli to lung

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102
Q

Patient w/ mitral stenosis develops low grade fever & negative blood cultures

A

Rheumatic fever history left patient with mitral stenosis –> now has another acute case of rheumatic fever

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103
Q

Strep bovis infection; what other conditions is patient most likely suffering from?

A 
Colonic cancer (or other GI malignancy)
Bacteremia
Endocarditis
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104
Q

Culture-negative endocarditis; valvular vegetations present; organisms don’t grown on standard blood culture

A

HACEK organisms

Haemophilus
Actinobacillus
Cardiobacterium
Eikenella
Kingella
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105
Q

Symptoms of S. aureus caused bacterial endocarditis

A

Shaking chills (rigors), high fever, dyspnea on exertion, malaise

Right sided endocarditis –> septic embolization into lungs –> pulmonary abscess

Left sided endocarditis –> heart failure, sepsis, septic embolization to brain & end organs

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106
Q

What conditions prevent gallstone formation?

A

Low cholesterol levels
High bile acid levels

Phosphatidylcholine – allows cholesterol to be more soluble

Obtain cholesterol by 1) absorption & 2) hepatic synthesis of cholesterol via HMG-CoA reductase

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107
Q

How is cholesterol transformed to soluble bile and excreted?

A

Free cholesterol converted to cholic and chenodeoxycholic acids (bile acids)

Bile acids conjugated –> glycine or taurine –> bile salts –> secreted into bile canaliculi

Phosphatidylcholine makes cholesterol more soluble

Gallbladder hypomotility & more cholesterol –> precipitation of gallstones

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108
Q

Transmural inflammation in Crohns leads to?

A

Strictures: due to chronic inflammation –> edema & fibrosis –> narrowed lumen (strictures)

Fistulas: inflammation + necrosis –> ulcer formation –> ulcer penetrates entire intestinal wall (transmural) –> formation of fitula

*Ulcerative colitis: damage limited to mucosa and submucosa

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109
Q

Toxic megacolon: complication of Crohn’s, UC, both?

A

Toxic megacolon affects both Crohn’s and UC; neuromuscular degeneration of intestinal wall –> rapid dilation –> bowel perforation

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110
Q

Worst prognostic factor for patient suffering from alcohol-induced liver damage?

A

Hypoalbuminemia & prolonged prothrombin time (PT)
both indicate decrease in hepatic function = liver failure

alcoholic steatosis & alcoholic hepatitis (reversible)
alcoholic cirrhosis (irreversible)

cirrhosis = hepatocellular injury + loss of hepatic function

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111
Q

Elevated alkaline phosphatase seen in bone disease; where else in GI/renal disorders?

A

Biliary disease has elevated alkaline phosphatase

use serum GGT to determine biliary disease (as opposed to bone disease)

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112
Q

Liver fibrinogen synthesis increase/decrease in liver failure?

A

Liver fibrinogen is coagulation factor and acute phase protein – increase in response to infection/acute inflammation

Liver failure failure = loss of hepatic function to make coagulation factors = LOW liver fibrinogen

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113
Q

Idiopathic membranous nephropathy - what molecular markers is it associated w/?

A

IgG4 antibodies to phospholipase A2 receptor (found in podocytes)

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114
Q

Minimal change disease; mechanism of pathogenesis?

A

abnormal T cell production –> glomerular permeability –> fusion of foot processes –> proteinuria

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115
Q

Mixed cryoglobulinemia

A

IgM deposition in glomerulus –> basement membrane thickening & cellular proliferation

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116
Q

Renal disease caused by multiple myeloma

A

Deposition of light chains (cast nephropathy)

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117
Q

SLE results in what type of glomerular disease?

A

Membranous GN

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118
Q

Hypocalcemia: what happens to PTH, Calcitonin, 1,25-dihydrocholecalciferol (active vit D)?

A

Increase PTH
Decrease calcitonin
Increase 1,25-

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119
Q

Chronic renal disease: what happens to PTH, Calcitonin, 1,25-dihydrocholecalciferol (active vit D)?

A

“Secondary hyperparathyroidism”

Increase PTH
Increase calcitonin (kidneys can’t excrete calcitonin)
Decrease 1,25-

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120
Q

Hypoparathyroidism: what happens to PTH, Calcitonin, 1,25-dihydrocholecalciferol (active vit D)?

A

Decrease PTH
Decrease calcitonin
Decrease 1,25-

121
Q

Administer calcium gluconate: what happens to PTH, Calcitonin, 1,25-dihydrocholecalciferol (active vit D)?

A

Decrease PTH
Increase calcitonin
Decrease 1,25-

122
Q

Autosplenectomy

A

Sickle cell anemia patients

Vaso-occlusive crises –> repeated splenic infarctions –> shrunken, discolored, fibrotic spleen

Brownish discoloration (hemosiderosis) due to extensive sickled RBCs by splenic macrophags (extravascular hemolysis)

123
Q

Symptoms of sickle cell crises

A

abdominal pain resolved w/ analgesics + hydration

acute chest syndrome – vaso-occlusive crisis localized to pulmonary vasculature (precipitated by pulmonary infection)

124
Q

Intrasplenic lipid accumulation

A

Lysosomal lipid storage disorders (Gaucher’s disease)

125
Q

Ischemic ATN is most likely a result of damage to which parts of renal tubules?

A

Proximal tubules
Thick ascending limb of loop of Henle

Ischemic ATN = most common cause of intrinsic renal failure (acure renal failure) in hospitalized pts.

Muddy brown casts seen

126
Q

Relationship btwn pt. hospitalized after MI and muddy brown casts seen in urine soon after?

A

Low cardiac output (cardiac arrest) –> ATN –> increased BUN and creatining & oligouria

Proximal tubules & thick ascending limb of loop of Henle most susceptible to ischemia (ATP-consuming activities of ion transport)

127
Q

Histology of ATN

A

Flattening of epithelial cells; loss of brush border in proximal tubular cells –> cell necrosis –> denudation of tubular basement membrane

128
Q

Renal papillary necrosis: disease associations

A

Diabetes
Analgesic nephropathy
Sickle cell disease
Severe obstructive pyelonephritis

129
Q

Hemolytic anemia + vasoocclusion in sickle cell anemia; what symptoms do you see w/ hemolytic anemia?

A

Jaundice - due to unconjugated hyperbilirubinemia (yellow sclera)
Pigmented gallstones
Depressed RBC indices

Painful crises – bone ischemia/necrosis –> Staph & Salmonella osteomyelitis

130
Q

Autosplenectomy - predisposed to infections w/ encapsulated organisms: which are?

A 
GBS
H. influenzae
S. pneumoniae
M. meningitidis
S. typhi
131
Q

Splenic sequestration

A

Young children w/ sickle cell
Vasooclussion –> splenic pooling of RBCs

Medical emergency!!
Marked decrease in Hgb, rapidly enlarging spleen, possible hypovolemic shock

132
Q

Pt. with sickle cell shows marked macrocytosis; why?

A

Megaloblastic anemia – impaired DNA syntheis
Lack of folic acid or vitamin B12

Hemolytic anemias predisposed to develop folic acid deficiency b/c of increase RBC turnover (use up all folic acid for DNA synthesis to make new RBCs)

Low folic acid –> impaired DNA synthesis BUT RNA synthesis is not affected –> cytoplasmic contents accumulate (cells swell and get bigger) but can’t divide (bc lack DNA material) –> macrocytosis

133
Q

Most common hepatobiliary disease in sickle cell patients

A

Pigmented gallstones

Liver-associated macrocytosis = alcoholic liver disease

134
Q

26 yr old Caucasian female w/ diplopia - resolves spontaneously; has problems adducting left eye when patient looks right; disease association?

A

Internuclear ophthalmoplegia (MLF syndrome) – MULTIPLE SCLEROSIS

loss of coordination of lateral conjugate gaze
decreased/blurred vision, central scotoma, monoocular blindness, painful eye movements

Sensory loss, arm/leg weakness, cerebellar symptoms (clumsiness, ataxia) also seen

135
Q

Pathogenesis of multiple sclerosis

A

Sclerotic plaques – appear anywhere in the white matter

Plaques form due to demyelination of axons

Changes of white matter seen inside plaques:
Axon demyelination
Depletion of oligodendrocytes (can’t make more myelin)
Accumulation of lipid-laden macrophages (chew up products of myelin breakdown)
Fibrillary astrocytosis (astrocyte proliferation reaction to injury)
Infiltration by lymphocytes & monocytes

136
Q

Synaptic transmission failure

A

Myasthenia gravis

Autoantibodies to Ach receptors

137
Q

Rapidly growing mass in liver of Australian immigrant; mass is damaged during surgery - what is patient at risk for; what is Rx?

A

Anaphylaxis

Mass is due to Echinococcus granulosus –> forms hydatid cysts
Rupture of cyst contents –> anaphylactic shock
Aspiration of cysts contraindicated.

Larvae implant in capillaries –> inflammatory rxn –> monocytes and eosinophils

Hydatid cyst is encapsulated & calcified; contains flui and budding cells

Rx: surgery, chemo, mebendazole/albendazole

138
Q

Flat yellow spots on inner surface of aorta; what are these? What can these spots progress to?

A

Fatty streaks: lipid-filled foam cells that have entered intima through injured, leaky endothelium; foamy appearance due to intracellular lipid-containing phagolysosomes

(foam cells derived from macrophages & smooth muscle cells that have engulfed LDL)

fatty streaks can be seen in aortas of children <1yr old – can progress to form atheromatous plaques, but NOT ALL progress to advanced atherosclerotic plaques

139
Q

Alternate pathway for blood to return to heart bypassing the IVC

A

Hemiazygous vein
Azygous vein
Accessory hemiazygous vein

140
Q

Urease activity test to detect what organism?

A

H. pylori –> duodenal ulcer

Uses urease to degrade urea into CO2 and NH3

141
Q

Best detection of congenital heart diseases (VSD, patent foramen ovale)

A

Echocardiogram

142
Q

Best detection of bronchiectasis

A

High resolution chest CT

143
Q

Patient has hyperacusis; pathology?

A

Stapedius n. (CNVII) innervates stapedius m. –> paralysis of m. allows wider oscillation of stapes –> increased sensitivity to sound

144
Q

Prolonged exposure to loud noises causes loss of hearing; how?

A

Damage to stereociliated hair cells of organ of Corti

145
Q

Damage to what structures causes conductive hearing loss?

A

Rupture of tympanic membrane

Defects of middle ear ossicles

146
Q

Pathway of sound transmission to organ of Corti

A

Sound –> middle ear via tympanic membrane

Vibration –> oval window via ossicles

Oval window vibration –> basilar membrane vibration –> hair cell cilia bends against tectorial membrane

Hair cells bending –> oscillating hyperpolarization & depolarization of auditory nerve

147
Q

Carotid sinus baroreceptors & Aortic arch baroreceptors – what innervates them? What occurs once receptors stimulated?

A

Carotid sinus - Glossopharyngeal n.
Aortic arch - Vagus n.

Stimulation of stretch receptors –> vasodilation, decrease in HR and contractility, decrease in BP

148
Q

Spoon nails, dysphagia; what deficiency is present?

A

Iron deficiency anemia

esophageal web = Plummer-Vinson
microcytic hypochromic RBCs

rx: give iron preparation

149
Q

Deficiency in vitamin B12 or folic acid – what kind of anemia occurs?

A

Megaloblastic anemia

150
Q

Deficiency in pyridoxine

A

Pyridoxine required as cofactor for 1st step in heme synthesis

Decreased heme synthesis –> microcytic, hypochromic pyridoxine-responsive anemia (sideroblastic anemia)

151
Q

Alveolar cells containing golden cytoplasmic granules –> dark w/ Prussian blue staining; what is pathology?

A

Prussian blue stains for IRON

Pt. has “heart failure cells” - macrophages that have engulfed a lot of RBCs in pulmonary circulation (due to pulmonary congestion/edema secondary to chronic LEFT heart failure)

152
Q

Turner’s syndrome: levels of FSH/LH?

A

Elevated LH and FSH – no negative feedback due to ovarian failure (no breast development, no menarche)

Small chance that some women can have spontaneous puberty –> possibility for pregnancy

Most can only become pregnant w/ donor oocytes - will need supplemental estrogen/progesterone to prepare and maintain uterine lining

153
Q

Fibroadenoma

A

most common benign tumor of breast
occur in multiples, in both breasts
increase in size during luteal phase of menstrual cycle/lactation
freely moveable, palpable mass (young)

cellular, myxoid stroma that encircles epithelium-lined glandular and cystic spaces

154
Q

Ductal carcinoma in situ (DCIS)

A

precancerous breast lesion
malignant clonal cell proliferation (contained by surrounding ductal BM)
Myopethelial (basal) layer preserved/uninvolved

155
Q

Paget disease of nipple

A

Malignant cells spread from superficial DCIS into nipple skin (does not cross BM)

unilateral erythema and scale crust around nipple

156
Q

Sclerosing adenosis

A

Central acinar compression/distortion (by surrounding fibrotic tissue) and peripheral ductal dilation

common finding in fibrocystic change

157
Q

Mammary duct ectasia

A

Ductal dilation, inspissated breast secretions

Chronic granulomatous inflammation in periductal and interstitial areas

158
Q

Medullary carcinoma

A

Solid sheets of vesicular, pleomorphic, mitotically active cells w/ significant lymphoplasmacytic infiltrate around/within tumor – pushing, noninfiltrating border

159
Q

Temporal arteritis

A

Inflammation in MEDIUM-sized and LARGE arteries – mononuclear infiltrates & multinucleated giant cells = “giant cell vasculitis”

Granulomas in media of arteries

Narrowing of vessel –> decrease in blood supply to perfused areas:

1) headache
2) craniofacial pain (jaw & tongue claudication) – while chewing
3) polymyalgia rehumatica – neck, torso, shoulder, pelvic girdle pain & morning stiffness; fever, fatigue, weight loss may also occur
4) sudden vision loss (monocular)

160
Q

Reversal treatment for ingestion of rat poison

A

Pts. susceptible to GI bleeding (no coagulation factors)

Give fresh frozen plasma and vitamin K1

161
Q

Hamartoma

A

Excessive growth of tissue type native to organ of involvement

162
Q

Most common benign lung tumor

A

Hamartoma (pulmonary chondroma)
“coin lesion” in pts. 50-60yrs old

Incidental finding on CXR – “popcorn calcifications”

contain islands of mature hyaline cartilage, fat, smooth muscle & clefts lined by respiratory epithelium

163
Q

Type of lung cancer w/ neuroendocrine markers

A

Small cell carcinoma of the lung

164
Q

Keratin pearls characteristic of what type of malignancy?

A

Squamous cell carcinoma

165
Q

Membranous GN

A

Nephrotic

Generalized edema
Heavy proteinuria (>3.5g/day)
Hypoalbuminemia
Hyperlipidemia

166
Q

IgA nephropathy (Berger disease)

A

Children/young adults
Painless hematuria
2-3 days after upper respiratory infection

(contrast w/ Post-STREP GN which occurs 2-3 weeks after upper respiratory or skin infection; anti-streptolysin O antibody; low C3 levels)

167
Q

Squamous cell carcinomas of bladder

A

RARE

Associated w/ Schistosoma haematobium infection

168
Q

Urothelial carcinoma

A

Formerly transitional cell carcinoma
makes up 90% of bladder cancers

Painless gross hematuria
Tumor penetration of bladder wall = major prognostic indicator
(carcinoma in situ - confined to epithelium)

169
Q

Vitelline duct connects what two structures; when it it obliterated? What condition results if duct persists?

A

Midgut lumen w/ yolk sac;
Obliterates 7th week

Persistence of vitelline duct = connection between intestinal lumen + umbilicus

Meckel diverticulum most common vitelline duct anomaly: partial closure of vitelline duct –> patent portion attached to ileum (fibrous band may connect tip of Meckel’s diverticulum w/ umbilicus) – can present w/ rectal bleeding/intestinal obstruction

170
Q

Umbilical hernia

A

Weakness of abdominal wall at umbilicus - midline protrusions covered by skin

171
Q

Omphalocele

A

Midline defect in abdominal wall - incomplete closure – abdominal organs protrude through defect covered w/ peritoneal sac

172
Q

Facor XIII deficiency

A

clot instability –> delayed, recurrent bleeding after trauma

173
Q

Protein C acts w/ Protein S to inactivate what factors?

A

Factor V and factor VIII

Inability to inhibit Factor V and VIII –> procoagulation state
Factor V Leiden mutation –> procoagulation (bc factor V can’t be inactivated)

174
Q

Factor X activates thrombin; what does thrombin activate?

A

Thrombin activates factor V, VIII, and XI

175
Q

ALS is combined UMN and LMN lesions: what structures involved?

A

Loss of neurons in anterior horn (LMN) - muscle weakness & atrophy

Degeneration/atrophy of lateral corticospinal tracts (UMN) - spasticity & hyperreflexia

176
Q

Treatment of ALS

A

Riluzole

MOA: decreases glutamate release

177
Q

What structures are damaged in poliomyelitis?

A

Enteroviral infection damages motor neurons in anterior horn –> LMN disease

Flaccid paralysis, atrophy, areflexia, fasciculation

178
Q

Huntington disease; what structures are damaged?

A

Caudate nucleus + putamen

179
Q

Symptoms of Friedrich ataxia

A 
AR disorder -- children 5-15yrs old
Ataxia
hypertrophic cardiomyopathy
kyphoscoliosis
foot abdnormalities
diabetes mellitus
180
Q

Pathogenesis of Vitamin B12

A

Subacute combined degeneration of:

posterior & lateral spinal columns

181
Q

Tumor staging vs. grading

A

Stage - TNM system (tumor size, nodal involvement, metastasis)
Grade - differentiation (high = high cellularity, nuclear peomorphism, poorly differentiated)

182
Q

Tumor with high bromodeoxyuridine uptake; what does this indicate?

A

Bromodeoxyuridine = thymidine analog

Thymidine = nucleotide
High nucleotide uptake occurs in S phase (cells ready to divide)

Tumor that has high thymidine uptake = high grade tumor & poor prognosis

183
Q

Older female, weight loss, abdominal discomfort, jaundice, epigastric mass; what GI association?

A

Pancreatic cancer

184
Q

Low pitched holosystolic murmur heard best at left sternal border; accentuation w/ handgrip exercise; what is defect and why is it heard better w/ maneuver?

A

VSD

handgrip maneuver –> increases afterload (pressure in systemic circulation that LV & aorta have to pump against)

increased afterload (pressure) causes more blood to be shunted from LV to RV (lower pressure system) so the murmur is heard louder

185
Q

Early diastolic murmur heard better with handgrip exercise?

A

Aortic regurgitation

186
Q

Mid-systolic pulmonary ejection murmur

A

due to increased flow across pulmonic valve

187
Q

Incomplete obliteration of processus vaginalis = connection btwn peritoneum & scrotum; what are 2 conditions that can occur from this connection?

A

Hydrocele (fluid leakage)

Indirect inguinal hernia (abdominal contents herniate down)

188
Q

Enzyme missing in albinism

A

Tyrosinase (uses tyrosine as substrate for melanin)

189
Q

Enzyme missing in alkatonuria

A

Homogentisic acid oxidase

Accumulation of homogentisic acid in body that needs to be renally excreted

190
Q

Gene mutations associated w/ Alzheimer’s

A

APP (chromosome 21)
presenilin 1 & 2
apoE4

APP & presenilin mutations promote AB-amyloid formation

191
Q

Familial hypercholesterolemia

A

AD disorder

LDL receptor mutations – liver can’t take up LDL –> severe elevation in total cholesterol

192
Q

Hypertrophic cardiomyopathy mutation

A

AD trait

mutation in B-myosin heavy chain

193
Q

Renal artery stenosis often caused by atherosclerosis; what cells undergo hyperplasia/hypertrophy? Why?

A

JG cells: renal artery stenosis cause hypoperfusion –> renin production increased in order to prodce more ATII –> vasoconstriction & aldosterone release
(JG cells are modified smooth m. cells in afferent & efferent arterioles)

Macula densa cells - distal tubule: sense osmolarity/volume of urine –> transmit back to JG cells

194
Q

Damage to common peroneal n. – what structure is involved?

A

Fibular head – occurs from lateral blows to knee/leg cast

Pain/numbness of dorsum of foot
Weakness in dorsiflexion of foot (foot drop)
Weakness in eversion of foot

195
Q

Deep peroneal n. (action)

A

Sensory to region btwn 1st and 2nd digits

Anterior compartment of leg (dorsiflexors of foot and toes) – in conjuction w/ common peroneal n.

196
Q

Tibial n. (action)

A

Plantar flexion & inversion of foot

197
Q

Important structures coursing near medial malleolus

A

Anteriorly:
Saphenous n.
Great saphenous vein

Posteriorly:
Posterior tibial a.
Tibial n.

Tendons of:
flexor digitorum longus
flexor hallucis longus
tibialis posterior

198
Q

What type of gating does CFTR gene have?

A

ATP-gated

F508 mutation
chromosome 7

CFTR hydraes mucosal surfaces; promotes secretion from ducts of exocrine pancrease; hypotonic sweat

Transport through eccrine ducts –> salt removed from ductal lumen
(hence chloride sweat test to detect elevated levels of salts)

199
Q

Where does cGMP-gated Na+ channels play a role?

A

Vision

200
Q

Persistent allantois remnant; what clinical features present?

A

Urine discharge from umbilicus

Allantois –> urachus (duct btwn bladder and urogenital sinus)

Failure of urachus to obliterate:

1) patent urachus – urine from umbilicus
2) vesicourachal diverticulum
3) urachal sinus (purulent discharge from umbilicus)
4) urachal cyst

201
Q

Physical manifestations of 47, XXX

A

Silent - normal females

some slightly decreased IQ scores

202
Q

Physical manifestations of 47, XXY

A

Tall male
gynecomastia, small testes, infertility

Klinefelter syndrome

203
Q

Physical manifestations of 47, XYY

A

Tall, severe acne

Delay in motor & language development

204
Q

Calcitriol

A

Active form of vitamin D converted in the KIDNEYS from 25-hydroxycholecalciferol

205
Q

Actions of calcitriol

A

Increase Ca2+ & phosphate absorption

206
Q

Chronic renal failure: what happens to PTH, Ca2+, phosphate, calcitriol?

A

Chronic renal failure = due to secondary hyperparathyroidism (high PTH)

Decreased renal parenchyma –> decreased calcitriol production

Lab values:
high PTH
low Ca2+
high phosphate
low calcitriol
207
Q

Why does Ca2+ levels decrease in chronic renal failure?

A

Decrease excretion of phosphate –> phosphate binds Ca2+ to form complexes –> less free Ca2+

208
Q

What two values always counteract each other in renal disease?

A

PTH and phosphate

High phosphate (lack of renal excretion) –> low Ca2+ –> elevate PTH

209
Q

Conn’s syndrome

A

primary hyperaldosteronism (zona glomerulosa hyperplasia)

primary adrenal hyperplasia
renal Na+ retention
excess K+ secretion
results in HTN and hypokalemia

Hypokalemia (muscle weakness); parethesias

210
Q

Weight gain + easy bruising; what adrenal condition?

A

Cushing’s syndrome
Glucocorticoid excess

Inhibited collagen and matrix GAG synthesis –> weaker skin/CT –> easy bruising

Zona fasiculata

211
Q

Hirsutism (excessive hair growth); what adrenal condition?

A

Androgen excess - adrenal tumor causing androgen hypersecretion
zona reticularis

212
Q

Diarrhea & flushing; what condition association?

A

Carcinoid syndrome

Serotonin(5-HT)-secreting tumor derived from neuroendocrine cells of GI tract

213
Q

Chronic myeloproliferative disorders (4)

A

CML - leukocytosis w/ marked left shift
Essential thrombocytosis - thrombocytosis
Polycythemia vera - erythrocytosis
Primary myelofibrosis - bone marrow fibrosis

214
Q

Mutation in myeloproliferative disorders (except for CML):

primary myelofibrosis, essential thrombocytois, essential thrombocytosis

A

JAK2 - tyrosine kinase (phosphorylation)

JAK2 causes persistent activation of STAT (signal transducers & activator of transcription)

215
Q

Primary myelofibrosis

A

atypical megakaryocytic hyperplasia

clonally expanded megakaryocytes activate fibroblast proliferation –> replacement of marrow space with collagen –> pancytopenia

peripheral smear: teardrop-shaped RBCs and nucleated RBCs

216
Q

Why is there hepatomegaly and splenomegaly in myelofibrosis?

A

Loss of bone marrow hematopoiesis –> extramedullary hematopoiesis

217
Q

Alcoholic is brought into hospital and stated on IV fluids + dextrose; develops ataxia, confusion, ophthalmoplegia. What enzyme is deficient in this patient’s TCA cycle?

A 
a-ketoglutarate dehydrogenase
cofactors (5):
Thiamine (deficient in alcoholics)
Lipoic acid
CoA
FAD
NAD+

conversion from a-ketoglutarate to succinyl-CoA

218
Q

Presence of unilateral fetal hydronephrosis –> what structure is possibly affected?

A

Ureteropelvic junction (junction btwn kidney & ureters) – last to canalize in fetal development

Transient hydronephrosis due to metanephros producing urine before canalization of ureteric bud is complete

219
Q

Origin of ureteric bud?

A

Metanephros

220
Q

Origin of collecting ducts, calices, renal pelvis, ureters, renal parenchyma?

A

Metanephros

221
Q

Most common cause of bacterial meningitis in adults of all ages?

A

Streptococcus pneumoniae

Lancet-shaped, Gram+ cocci in pairs

Increase neutrophils, decrease glucose, increase opening pressure

Pt. w/ confusion, severe headache, nausea, nuchal rigidity

222
Q

Pt. with Parkinsons complains of “freezing up” moments followed by episodes of not experiencing any rigidity; why?

A

Drug response is UNPREDICTABLE and NOT dose-dependent

“On-off”phenomenon:
Sudden loss of anti-Parkinsonian effects of levodopa (L-dopa) –> hypokinesis and rigidity; other times, levodopa works.

223
Q

What kind of pathology is associated w/ elevated conjugated bilirubin?

A

Hepatobiliary disease (cirrhosis or hepatitis) – bilirubin conjugates will reflux back to plasma when secretion of bilirubin into bile is slow

224
Q

What kind of pathology is associated w/ elevated Unconjugated bilirubin?

A

Increased bilirubin formation (hemolysis)

Slowing in bilirubin conjugation (Gilbert syndrome)

225
Q

Gilbert syndrome

A

UDP glucuronyl transferases decreased

Bilirubin glucoronidation decreased

226
Q

What is the association btwn Wilson’s disease and the liver?

A

Pt. younger than 30 yrs w/ unexplained chronic hepatitis (elevated AST and ALT)

Presence of ceruplasmin & urinary copper excretion
Kayser-Fleischer rings

227
Q

Superior mesenteric artery syndrome; what are predisposing factors? At what level does SMA leave aorta?

A

Transverse portion of duodenum gets entrapped btwn SMA and aorta –> partial intestinal obstruction

Predisposing factors:
Aortomesenteric angle decreased
Diminished mesenteric fat
Pronounced lordosis
Surgical correction of scoliosis
228
Q

What malignancies is Down syndrome associated w/?

A

AML
ALL
early Alzheimer

229
Q

Cause of Cushing’s syndrome: ACTH dependent vs ACTH independent

A

ACTH dependent:

a) Cushing’s disease - pituitary ACTH hypersecretion
b) Ectopic ACTH syndrome - paraneoplastic ACTH secretion

ACTH independent:
a) adrenal adenoma or carcinoma

230
Q

Embryonic aortic arch that ductus arteriosus derives from

A

6th

231
Q

Patent ductus arteriosus; what to keep it open; what to close it?

A 
PGE2 = maintain patency
Indomethacin = close PDA (usually ductus arteriosus closes after birth when partial pressures of O2 increase and PGE2 decrease)

“Machinery-like” continuous murmur, bounding pulse with thrill palpated over upper left sternal edge

After closure, known as ligamentum arteriosum

232
Q

How is gastric acid secretion by parietal cells in mucosal glands (fundus & body of stomach) stimulated?

A

1) Histamine –> H2 receptors –> increase intracellular cAMP
2) Acetylcholine –> M3 muscarinic receptors –> increase intracellular Ca2+

3) Gastrin –> gastrin receptor:
- - increase intracellular Ca2+ concentration
- - histamine synthesis & release by ECL (enterochromaffin-like cells) in stomach***

233
Q

What action does increased intracellular Ca2+ and cAMP have on gastric acid secretion?

A

Ca2+ increase (activates things)
cAMP activate protein kinases
BOTH lead to increased transport of H+ by H+K+ ATPase into gastric lumen

234
Q

Systemic mastocytosis (symptoms)

A

Mast cell proliferation –> increased histamine release –> gastric acid secretion increases

Gastric hypersecretion:
Inactivates pancreatic & intestinal enzymes –> diarrhea
Gastric ulcerations

Increase mast cells:
Syncope
Flushing
Hypotension --> Tachycardia
Bronchospasm
Pruritis, urticaria, dermatographism
235
Q

Pernicious anemia

A

Autoantibody to parietal cells/intrinsic factor

IF not produced –> vit. B12 not absorbed –> neurologic symptoms

236
Q

Substances that pancreatic tumors secrete:

A

1) Gastrin: ZE syndrome
2) Insulin: fasting hypoglycemia
3) Glucagon: diabetes mellitus & RASH*
4) Somatostatin: diarrhea, cholelithiasis, diabetes mellitus

237
Q

Opening snap (extra heart sound) shortly after S2 heart sound (AV close); Diastolic rumbling murmur after opening snap best heard at heart apex

A

Mitral stenosis

Diastolic rumbling murmur due to turbulent flow through stenottic mitral valve during LV filling

Extra heart sound (opening snap) due to abrupt halting of leaflet motion during mitral valve opening due to fusion of mitral valve leaflet tips

238
Q

Congenital torticollis

A

2-4 weeks old
Child prefers to hold head tilted to one side (cries when you try to move child’s nexk; could present w/ firm swelling in neck)

Due to malposition of head in utero or birth trauma (breech delivery) –> SCM m. injury & fibrosis

239
Q

What can bind to antithrombin? What does antithrombin do?

A

Unfractioned heparin and LMWH bind antithrombin –> antithrombin activity against Factor Xa

Unfractioned heparin able to bind to both antithrombin & thrombin –> allow antithrombin to inactivate thrombin

240
Q

Enoxaparin

A

LMWH

241
Q

Fondaparinux

A

Synthetic pentasaccharide factor Xa inhibitor

242
Q

Relationship of ureters w/ common/external iliac vessels and gonadal vessels?

A

Ureters:
course on TOP of psoas muscle
cross OVER common/external iliac vessels
cross UNDER gonadal vessels (ovarian vessels in females) - “water under the bridge”

enter true pelvis:
pass LATERAL to internal iliac vessels
pass MEDIAL to gonadal vessels as they enter true pelvis

243
Q

Ventromedial nucleus of hypotahalmus destroyed

A

Person eats more and becomes obese

ventromedial nucleus responsible for satiety

244
Q

Lateral nucleus of hypothalamus destroyed

A

Person starves (lateral nuclei responsible for hunger)

245
Q

Supraoptic and paraventricular nuclei destroyed

A

No release of ADH or oxytocin (later carried down to neurohypophysis)

246
Q

Anterior hypothalamic nuclei vs. posterior hypothalamic nuclei in relation to heat production

A

Anterior hypothalamic nuclei = vasodilation & sweating = COOL

Posterior hypothalamic nuclei = conserve heat, heat production = HOT

247
Q

What morphological change occurs in vessels in primary pulmonary HTN? What causes it?

A

Increase thickness of smooth muscles in arterioles (medial hypertrophy); fibrosis of intima

Inherited disease
Decreased apoptosis of endothelial and smooth muscles cells in pulmonary aa

Presents in women; dyspnea and exercise intolerance

248
Q

Rx for primary pulmonary HTN

A

Bosentan - endothelin-receptor antagonist
(endothelin vasoconstricts and stimulates endothelial proliferation) – blocking of endothelin –> decrease pulmonary arterial pressure (lessen RV hypertrophy)

249
Q

Pt. w/ chronic aortic stenosis (AS) experiences a. fib; what symptoms exacerbated? What could happen to venous pressures?

A

Reduced ventricular preload –> CO decrease –> dangerous systemic hypotension

Acute a. fib can also increase steady state pulmonary venous pressures sufficiently to cause acute pulmonary edema

250
Q

Hyperpyrexia (temp?); what should be done first?

A

Greater than 40C

Can lead to permanent brain damage

Give acetaminophen to kids (inhibits COX in CNS, decrease PGE synthesis); avoid ASA due to Reye syndrome

251
Q

Bacterial LPS can stimulate high fevers; what substances released to induce fever?

A

IL-1
IL-6
TNF

252
Q

What vessel is derived from the common cardinal veins?

A

SVC

253
Q

How does left heart failure lead to increased pulmonary arterial pressure?

A

LH failure –> backup of fluids in lungs –> pulmonary edema causes collapse of alveoli –> less ventilation –> VASOCONSTRICTION to shunt blood away from O2 poor areas –> pulmonary arterial HTN due to vasoconstriction –> increase afterload for right ventricle –> right heart failure

254
Q

Indirect vs. Direct vs. Femoral hernias

A

Indirect: male infants - LATERAL to inferior epigastric vessels; persistent processus vaginalis; failure of inguinal ring to close

Direct: older men; bulges from Hesselbach’s triangle; MEDIAL to inferior epigastric vessels; due to weakness of transversalis fascia

Femoral: women; protrudes from femoral ring; medial to femoral vessels; INFERIOR to inguinal ligament

255
Q

Hesselbach’s triangle

A

Inguinal ligament - inferiorly
Inferior epigastric vessels - laterally
rectus abdominus - medially
Transversalis fascia - floor of triangle

256
Q

Uncinate process of pancrease/major pancreatic duct - derived from?

A

Ventral bud

257
Q

Pancreas divisum

A

Dorsal & ventral buds don’t fuse

Clinically silent usually

Can predispose to recurrent pancreatitis; accessory duct drains majority of pancreas

258
Q

Middle meningeal a. vs. middle cerebral a.

A

Middle MENINGEAL - branch of MAXILLARY – branch of EXTERNAL carotid a.
can be ruptured due to trauma in area where frontal, parietal, temporal, and sphenoid bones meet –> EPIDURAL hematoma –> transtenorial herniation, palsy of CNIII

Middle CEREBRAL - branch of INTERNAL carotid a.
can lead to SUBARACHNOID or intracerebral hemorrhage

259
Q

What does a decrease in CO in CHF patients do to renin-angiotensin system?

A

Decrease CO –> low stretch in glomerular afferent arterioles –> renin-angiotensin system activation to bring BP up –> vasoconstriction –> increased arterial resistance and afterload (the pressure LV has to pump against)

260
Q

Where is angiotensinogen converted to angiotensin I?

A

Liver

261
Q

Systolic dysfunction

A

Decrease in myocardial contractility

262
Q

Diastolic dysfunction

A

Inability of heart to relax sufficiently to allow adequate filling of blood

263
Q

Zollinger-Ellison; why is it associated w/ diarrhea?

A

Hypersecretion of gastrin via pancreatic tumor

Peptic ulcers result

Diarrhea – due to inactivation of intestinal & pancreatic enzymes by EXCESSIVE GASTRIC ACID

264
Q

Polyarteritis nodosa (PAN)

A

Segmental TRANSMURAL NECROTIZING inflammation

Medium/small sized arteries

Necrotic tissue/immune complexes/complement/plasma proteins w/ smudgy eosinophilic deposit (fibrinoid necrosis) – immune complex vasculitis

265
Q

Buerger’s disease (thromboangiitis obliterans)

A

Thrombosing vasculitis - medium/small sized arteries

Tibial and radial arteries

Heavy smokers, onset prior to 35

266
Q

Takayasu arteritis

A

Giant cell (temporal) arteritis
Jaw claudication
Affects aortic arch
Females <40yrs

267
Q

Lesions present in primary vs. secondary vs. tertiary syphilis

A

Primary - painless ulceration: chancre

Secondary - Condyloma lata: gray wart-like growths

Tertiary - Gummas: painless, indurated granulomatous lesions

268
Q

Positive VDRL and pleocytosis in CSF: diagnosis?

A

Tertiary syphilis (neurosyphilis)

269
Q

Condylomata acuminata

A

HPV 6 and 11

270
Q

Sudden, painless, permanent monocular blindenss; pale retina & cherry red macula

A

Central retinal artery occlusion

271
Q

What major arteries does the central retinal artery branch off from?

A

Ophthalmic artery (branch from internal carotid artery)

Embolism (from a. fib or carotid artery stenosis) –> central artery occlusion

272
Q

Blurry vision, poor night vision, floaters, decreased peripheral vision; “cotton wool” exudates, “dot and blot” hemorrhages & new retinal vessel formation

A

Diabetic retinopathy

273
Q

Amaurosis fugax

A

Painless, transient, monocular vision loss due to small embolus to ophthalmic artery

274
Q

Temporal lobe infarcts - what vision changes occur?

A

Meyer’s loop affected –> CONTRA upper quadrantanopsia

275
Q

Large pituitary adenoma - what vision changes occur?

A

Bitemporal hemianopia

276
Q

Fall on outstretched, dorsiflexed hand; what wrist bone is affected?

A

Lunate dislocation

Lunate next to the scaphoid bone – both articulate w/ the radius –> avascular necrosis

277
Q

Relative polycythemia

A

Dehydration
Excessive diuresis

Decreased plasma volume
Normal RBC mass
Normal SaO2 & EPO level

278
Q

Primary polycythemia

A

Polycythemia vera

Increased plasma volume
Increased RBC mass
Decreased EPO**

279
Q

Secondary polycythemia

A

Physiologic - HYPOXIA:
Increased RBC mass (to transfer more O2)
Decreased SaO2 (hypoxic state)
Increased EPO

Inappropriate - EPO producing tumors:
Increased RBC mass
Increased EPO

280
Q

Crohn’s disease - association with kidney stones?

A

Crohn’s disease affects the terminal ileum - bile acids can’t be reabsorbed in terminal ileum –> bile acids lost in feces –> fat malabsorption

Increase in fat content –> fat binds Ca2+ (so Ca2+ can’t bind oxalate to be excreted) –> oxalate reabsorbed –> promotes urinary stone formation

281
Q

Lung physiology in patient w/ COPD

A

Increase TLC
Decrease FEV1/FVC
Decrease FVC
Increase RV

282
Q

Why is there increase in both TLC and RV in COPD?

A

Destruction of interalveolar walls –> decrease in lung elastic recoil & distal airspace enlargement

Increase TLC (air trapped in enlarged air spaces); Increase RV

Expiratory airflow obstruction – Decrease FEV1

Similar conditions to COPD:
Emphysema (smoking)
Bronchitis

283
Q

Lung physiology in patient with restrictive lung disease

A

Decrease TLC
Decrease FVC
Decrease RV
Increase FEV1/FVC

Increase elastic recoil
Decrease lung expansion

284
Q

Ligation of the superior thyroid artery; what muscle may become affected if the nerve near the superior thyroid artery is damaged?

A

Superior thyroid artery - branch of external carotid

Vagus n –> superior laryngeal n. and recurrent laryngeal n.

Superior laryngeal nerve –> CRICOTHYROID muscle & sensory innervation ABOVE vocal cords

Recurrent laryngeal n. –> all laryngeal m. except cricothyroid; sensory innervation BELOW vocal cords

285
Q

Paroxysmal breathlessness & wheezing; granule-containing cells with crystalloid bodies; what condition does the pt. have?

A

Allergic asthma

Granule containing cells = eosinophils
Crystalloid bodies = Charcot-Leyden crystals (contain eosinophil membrane protein)

286
Q

What factor activates eosinophils in pts. w/ chronic eosinophilic bronchitis?

A

IL-5 released by TH2 cells

287
Q

Differential diagnosis for decreased visual acuity & tunnel vision in low light (night blindness)

A
  1. Phenothiazines (antipsychotics/antihistamines) or chloroquine
  2. Vitamin A deficiency (could be due to biliary obstruction –> cholestasis –> fat soluble vitamin malabsorption)
  3. Congenital rubella, syphilis
  4. Diabetic retinopathy
288
Q

Crescendo-Decrescendo systolic murmur: what valve disease?

A

Aortic stenosis

Due to:
Degenerative (senile) calcinosis of aortic leaflets

289
Q

Mitral stenosis;

Mitral & aortic regurgitation – what disease association?

A

Rheumatic fever

290
Q

Cystic medial necrosis - what predisposing factors?

A

Cystic medial necrosis –> aortic dissection (abrupt severe chest pain) –> dilation of aortic root (aortic regurgitation)

291
Q

Broca’s aphasia; what area of brain affected?

A

Inferior frontal gyrus of dominant (usually left) hemisphere

292
Q

Precentral vs. Postcentral gyrus damage: effects?

A

Precentral gyrus (primary motor cortex): dysarthria (slurred/explosive speech); Broca’s deficit (usually left dominant hemisphere) often seen w/ right sided hemiparesis

Postcentral gyrus (primary somatosensory cortex): sensation loss to CONTRA body

293
Q

What nerve innervates muscles of mastication?

A

CNV3 (mandibular branch) - exits via foramen ovale

Masseter, medial pterygoid, temporalis m. = close jaw

Lateral pterygoid = opens jaw

294
Q

Child w/ white pupillary reflex; what malignancy? What associated conditions?

A

Retinoblastoma due to Rb gene mutation

Familial retinoblastoma –> osteosarcoma later in life

Rb gene mutations - usually associated w/ 2-Hit hypothesis

295
Q

Ewing sarcoma

A

Neoplasm of neuroectodermal cells

Long and flat bones of children/adolescents

296
Q

Most common malignant brain tumor of childhood

A

Medulloblastoma (arises in cerebellum)

297
Q

What causes proteolysis of factor V and VIII?

A

Protein C (actions of protein C increased by protein S)

Protein C and Protein S prevent thrombosis

298
Q

Radial nerve deficit; what symptoms? What fracture results in radial n. deficit?

A

C5-C8 and T1 innervates radial nerve
Skin of posterior arm & forearm
Extensor muscles of upper limb

Humerus fracture –> radial nerve affected –> wrist drop

299
Q

Damage to what nerve affects opposition of thumb?

A

Median n.

STEP1 - TLe (8 decks)

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