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)
2
Q
IgA nephropathy;
purpuric lesions on extensor surfaces (arms, legs, butt);
abdominal pain/vomiting/intestinal bleeding/intussusception
A
Henoch-Schonlein disease
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
4
Q
VIPomas
A
excess water, Na+, and K+ loss in stool
inhibits gastric acid secretion
watery diarrhea
hypokalemia
achlorhydria
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
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
7
Q
Intraperitoneal structures that can cause hemoperitoneum
A
transverse colon, liver, spleen
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
9
Q
Cardiac defect in Down syndrome
A
ASD
fixed splitting of second heart sound
VSD
holosystolic murmur
10
Q
Cardiac defect in rheumatic heart disease
A
mitral stenosis
mid-diastolic, low-pitched rumbling murmur (may begin w/ opening snap)
11
Q
Cardiac defect in Marfan and Ehlers-Danlos
A
MVP
Mid-systolic click, late systolic murmur
12
Q
Cardiac defect in premature infants w/ respiratory distress syndrome
A
PDA
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
14
Q
Prinzmetal’s (variant) angina – treatment?
A
Coronary artery vasospam
Nitroglycerin (or other vasodilators)
15
Q
Acute transmural myocardial infarction (MI)
A
peaked T waves (localized hyperkalemia)
ST segment elevation
Q waves
anterior: V1-V3
I, aVL: lateral
16
Q
Stable angina
A
Stable atheromatous lesion WITHOUT thrombus
Obstructs greater than 75% of coronary artery lumen
17
Q
Bronze diabetes
A
Skin hyperpigmentation
Diabetes mellitus
Pigment cirrhosis w/ hepatomegaly
Associated w/ late stage hemochromatosis
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
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
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
21
Q
Presystolic sound immediately preceding first heart sound - what condition?
A
S4 gallop is presystolic sound immediately before S1
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
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
24
Q
What does S1 and S2 represent?
A
S1 = closing of AV valves S2 = closing of semilunar valves (pulmonic and aortic)
25
Cutaneous melanoma gene mutation
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
26
Glioblastoma causes overexpression of:
growth factors (PDGF, EGFR, IGF, TGF)
27
Taste & sensory sensation to anterior 2/3 and poster 1/3 of tongue?
anterior 2/3:
Sensory (pain) - CNV3 (mandibular branch of trigeminal)
Taste - CNVII (facial)
posterior 1/3:
sensory & taste - CNIX
28
Hydrocele development - cause?
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
29
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?
Spider angiomas
Dependent on estrogen
30
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?
Cavernous hemangiomas - associated w/ von Hippel-Lindau disease
31
Lymphatic cysts lined by thin endothelium - benign tumors present at birth located on neck and lateral chest wall; what disease associations?
Turner syndrome
| Down syndrome
32
Cherry hemangioma vs. Strawberry hemangiomas
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
33
Treatment for diabetic ketoacidosis
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
34
Symptoms of DKA
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
35
Opioid antagonists uses
treats opioid overdoses --> respiratory acidosis due to hypoventilation
36
Winter's formula
PaCO2 = (1.5 x HCO3-) + 8 +- 2
37
Winter's formula (when to use it?)
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
38
Elevated AFP
Hepatocellular carcinoma (HCC)
39
Elevated CEA
Colorectal cancer
40
Elevated CA-125
Ovarian cancer
```
other cancers:
endometriosis
lung
breast
pancreatic* cancer
```
41
Acid phosphatase elevation
Secondary prostate tumor marker antigen
also in patients w/ active osteoclast-initiated bone resorption
42
hCG
suspected gestational trophoblastic disease
43
Physical manifestations of Marfan's
spinal scoliosis and/or kyphosis
lens dislocation
cystic medial degeneration of aorta --> aortic dissection (cause of death)
defect in glycoprotein fibrillin-1
44
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)
Li-Fraumeni syndrome
several cancers appear
cancers occur at young age
cancers appear several times throughout lifetime
45
Follicular lymphoma
Bcl-2 (proto-oncogene) overexpression --> antiapoptosis of tumor cells (immortality)
t(14;18)
46
CML
Bcr-abl
| t(9;22) Philadelphia chromosome
47
Burkitt's lymphoma
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
48
Patient hospitalized for acute MI dies 3-7 days after onset of MI; why?
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
49
Most common cause of in-hospital death due to MI
LV failure/cadiogenic shock
50
Frequent complication of cornary artery fibrinolysis (to get rid of clot)
Systemic bleeding, especially intracranial hemorrhage
51
Joint pain + cardiac murmur
rheumatic heart disease
antibodies against M-proteins of Streptoccocci
52
Nasal ulcer + hematuria
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
53
malar rash + pleural effusion
SLE
circulating immune complex nephritis
dsDNA & anti-Sm
54
dysphagia & sclerodactyly
CREST syndrome
calcinosis, Raynaud's, esophageal dysmotility, scerodactyly, telangiectasia
anti-centromere antibodies
55
Pt. w/ choking spells, dysphagia, coughing; recurrent pneumonia; what could be cause of choking spells?
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
56
Congenital hydrocele
Processus vaginalis remains patent --> fluid from peritoneum accumulates in scrotum
normal development: testis descends through inguinal canal; peritoneal tissue also descend = processus vaginalis
57
Young black woman; malaise, cough, cutaneous findings (including erythema nodosum - painful shin nodules); bilateral hilar lymphadenopathy; non-caseating granuloma
Sarcoidosis
elevated ACE levels
chronic restrictive granulomatous interstitial lung disease
58
Cellular immunity
T -cell response (NOT B-cell response)
Vaccines generally stimulate B-cells --> memory B lymphocytes & humor immunity
59
Wrist drop -- radial nerve mononeuropathy; causes?
Compression
Trauma
Diabetic vasculopathy
Vasculitis
60
Antibodies against neutrophil myeloperoxidase
p-ANCA (perinuclear staining antineutrophil cytoplasmic antibodies)
microscopic polyangiitis or Churg-Strauss vasculitis
61
Churg-Strauss
```
idiopathic systemic vasculitis
adult-onset asthma
eosinophilia
mono or polyneuropathy
migratory/transient pulmonary infiltrates
paranasal sinus abnormalities
```
62
Mesothelioma
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
63
Bronchioalveolar carcinoma
type of adenocarcinoma
PERIPHERY of lung (peripheral mass) - pneumonia like consolidation
distribution along alveolar septa
64
Small cell carcinoma
Major bronchi
hilar mass
Neuroendocrine markers - chromogranin & synaptophysin+
65
Squamous cell carcinoma
hilar mass
keratinization & intercellular bridges
cavitary lung lesion
66
Mantle cell lymphoma
t(11;14)
Immunoglobulin heavy chain (IgH) is on 14
cyclin D1 -- promoter of G1 to S-phase transition
67
Mutations of DNA repair enzymes
```
Breast cancer
Ovarian cancer
Lynch syndrome
Ceroderma pigmentosum
Fanconi anemia
```
68
Hemophilia A
X-linked (affects males)
factor VIII deficiency
prolonged PTT
hemorrhage into joints (hemarthrosis); prolonged bleeding after surfical procedures
69
Hemophilia B
factor IX deficiency
Christmas disease
prolonged PTT
70
Protein C deficiency
Lack of protein C --> procoagulation
| Suffer from recurrent deep venous thromboses
71
Deficiency in factors II, V, VII, X, & fibrinogen; effect seen in PT or PTT?
PT prolongation
| Warfarin -- blocks coagulation factors --> also causes PT prolongation
72
Mucocutaneous bleeding (recurrent epistaxis, gingival hemorrhage); what type of blood disorder?
Defect in platelets --> bleeding time prolongation
also seen in vWF disease and NSAIDs
73
What does thrombin time measure?
rate of conversion of fibrinogen --> fibrin
| thrombin time prolonged when there is deficiency/defective fibrinogen
74
Low haptoglobin is an indication of?
Hemolytic anemia
75
Hirschsprung disease
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
76
Pseudogout vs. gout
Pseudogout:
Knee joint
Rhomboid-shaped positive birefringent
Calcium pyrophosphate crystals
```
Gout:
1st metatarsophalangeal joint
Monosodium urate (salt of uric acid)
```
77
Calcific tendonitis
Calcium hydroxyapatitie crystals in periarticular soft tissue (esp. tendons)
Rotator cuff usually affected
78
Xanthomas
Soft tissue deposits of cholesterol
Hyperlipidemia
Yellow papules on knees, elbows, tendon insertion sites
79
Reye syndrome
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
80
The only disease in which ASA is administered to children under 16yrs
Kawasaki disease
Vasculitis of medium-sized arteries (affects children)
Conjunctivitis
Cervical lymphadenopathy
Periungal desquamation (fingers)
Mucocutaneous changes (strawberry tongue)
81
Centrilobular congestion
Occurs in liver of patients w/ right sided heart failure
82
Primary biliary cirrhosis
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
83
Male w/ UC presents w/ fatigue, high alkaline phosphatase
Primary sclerosing cholangitis
84
Preload
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
85
Afterload
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
86
Why is the pO2 in the left atrium lower than in the pulmonary capillaries?
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
87
Determining acid-base disturbance; normal pCO2; normal HCO3-?
pH > 7.45 alkalosis
pH < 7.35 acidosis
pCO2 normal = 40
HCO3- normal = 24
88
Effect of heroin overdose on blood gases?
Heroin suppresses respiratory centers --> hypoventilation w/ rentention of CO2 (acute phase)
In chronic respiratory acidosis - kidneys compensate by excreting H+ and reabsorbing HCO3-
89
When does contraction alkalosis occur? Lab findings?
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)
90
Blood gas findings in diabetic ketoacidosis?
low pH, low HCO3- (to neutralize accumulated acidic products) leading to metabolic acidosis, low pCO2 (hyperventilation or Kussmaul breathing to compensate for acidosis)
91
How does brown fat generate heat?
Have several intracytoplasmic fat droplets
Contain more mitochondria than white adipose
Uncouples oxidative phosphorylation with protein thermogenin
92
Restrictive cardiomyopathy
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
93
Oral thrush - what types of popn. suffer from this infection?
```
Denture wearers
Diabetics
Immunosuppressed patients (HIV)
Steroid use
Antibiotics, chemotherapy
```
94
Luekoplakia
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
95
Animal contact -- fungal infection
Microsporum species causing dermatophytosis
96
Varicose veins - causes and complications?
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!)
97
Cavernous hemangiomas
Soft blue compressible masses
| on light microscopy -- large dilated vascular spaces
98
Presence of schistocytes
```
Microangiopathic hemolytic anemia (TTP, HUS, DIC)
Mechanical damage (prosthetic valve)
```
99
HUS vs. TTP; similarities and differences?
Similar symptoms: fever, neurologic manifestations, renal failure, thrombocytopenia, microangiopathic hemolytic anemia
TTP: adults - neurological symptoms predominate
HUS: children - renal involvement predominate
100
K+ sparing diuretics; location of action?
Spironolactone
Amiloride
Triamterene
K+ sparing diuretics work at collecting duct
101
Right-sided endocarditis
S. aureus -- in IV drug users
Perforations to heart valves, rupture of chordae tendinae, septic emboli to lung
102
Patient w/ mitral stenosis develops low grade fever & negative blood cultures
Rheumatic fever history left patient with mitral stenosis --> now has another acute case of rheumatic fever
103
Strep bovis infection; what other conditions is patient most likely suffering from?
```
Colonic cancer (or other GI malignancy)
Bacteremia
Endocarditis
```
104
Culture-negative endocarditis; valvular vegetations present; organisms don't grown on standard blood culture
HACEK organisms
```
Haemophilus
Actinobacillus
Cardiobacterium
Eikenella
Kingella
```
105
Symptoms of S. aureus caused bacterial endocarditis
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
106
What conditions prevent gallstone formation?
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
107
How is cholesterol transformed to soluble bile and excreted?
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
108
Transmural inflammation in Crohns leads to?
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
109
Toxic megacolon: complication of Crohn's, UC, both?
Toxic megacolon affects both Crohn's and UC; neuromuscular degeneration of intestinal wall --> rapid dilation --> bowel perforation
110
Worst prognostic factor for patient suffering from alcohol-induced liver damage?
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
111
Elevated alkaline phosphatase seen in bone disease; where else in GI/renal disorders?
Biliary disease has elevated alkaline phosphatase
| use serum GGT to determine biliary disease (as opposed to bone disease)
112
Liver fibrinogen synthesis increase/decrease in liver failure?
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
113
Idiopathic membranous nephropathy - what molecular markers is it associated w/?
IgG4 antibodies to phospholipase A2 receptor (found in podocytes)
114
Minimal change disease; mechanism of pathogenesis?
abnormal T cell production --> glomerular permeability --> fusion of foot processes --> proteinuria
115
Mixed cryoglobulinemia
IgM deposition in glomerulus --> basement membrane thickening & cellular proliferation
116
Renal disease caused by multiple myeloma
Deposition of light chains (cast nephropathy)
117
SLE results in what type of glomerular disease?
Membranous GN
118
Hypocalcemia: what happens to PTH, Calcitonin, 1,25-dihydrocholecalciferol (active vit D)?
Increase PTH
Decrease calcitonin
Increase 1,25-
119
Chronic renal disease: what happens to PTH, Calcitonin, 1,25-dihydrocholecalciferol (active vit D)?
"Secondary hyperparathyroidism"
Increase PTH
Increase calcitonin (kidneys can't excrete calcitonin)
Decrease 1,25-
120
Hypoparathyroidism: what happens to PTH, Calcitonin, 1,25-dihydrocholecalciferol (active vit D)?
Decrease PTH
Decrease calcitonin
Decrease 1,25-
121
Administer calcium gluconate: what happens to PTH, Calcitonin, 1,25-dihydrocholecalciferol (active vit D)?
Decrease PTH
Increase calcitonin
Decrease 1,25-
122
Autosplenectomy
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
Symptoms of sickle cell crises
abdominal pain resolved w/ analgesics + hydration
| acute chest syndrome -- vaso-occlusive crisis localized to pulmonary vasculature (precipitated by pulmonary infection)
124
Intrasplenic lipid accumulation
Lysosomal lipid storage disorders (Gaucher's disease)
125
Ischemic ATN is most likely a result of damage to which parts of renal tubules?
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
Relationship btwn pt. hospitalized after MI and muddy brown casts seen in urine soon after?
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
Histology of ATN
Flattening of epithelial cells; loss of brush border in proximal tubular cells --> cell necrosis --> denudation of tubular basement membrane
128
Renal papillary necrosis: disease associations
Diabetes
Analgesic nephropathy
Sickle cell disease
Severe obstructive pyelonephritis
129
Hemolytic anemia + vasoocclusion in sickle cell anemia; what symptoms do you see w/ hemolytic anemia?
Jaundice - due to unconjugated hyperbilirubinemia (yellow sclera)
Pigmented gallstones
Depressed RBC indices
Painful crises -- bone ischemia/necrosis --> Staph & Salmonella osteomyelitis
130
Autosplenectomy - predisposed to infections w/ encapsulated organisms: which are?
```
GBS
H. influenzae
S. pneumoniae
M. meningitidis
S. typhi
```
131
Splenic sequestration
Young children w/ sickle cell
Vasooclussion --> splenic pooling of RBCs
Medical emergency!!
Marked decrease in Hgb, rapidly enlarging spleen, possible hypovolemic shock
132
Pt. with sickle cell shows marked macrocytosis; why?
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
Most common hepatobiliary disease in sickle cell patients
Pigmented gallstones
Liver-associated macrocytosis = alcoholic liver disease
134
26 yr old Caucasian female w/ diplopia - resolves spontaneously; has problems adducting left eye when patient looks right; disease association?
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
Pathogenesis of multiple sclerosis
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
Synaptic transmission failure
Myasthenia gravis
Autoantibodies to Ach receptors
137
Rapidly growing mass in liver of Australian immigrant; mass is damaged during surgery - what is patient at risk for; what is Rx?
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
Flat yellow spots on inner surface of aorta; what are these? What can these spots progress to?
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
Alternate pathway for blood to return to heart bypassing the IVC
Hemiazygous vein
Azygous vein
Accessory hemiazygous vein
140
Urease activity test to detect what organism?
H. pylori --> duodenal ulcer
| Uses urease to degrade urea into CO2 and NH3
141
Best detection of congenital heart diseases (VSD, patent foramen ovale)
Echocardiogram
142
Best detection of bronchiectasis
High resolution chest CT
143
Patient has hyperacusis; pathology?
Stapedius n. (CNVII) innervates stapedius m. --> paralysis of m. allows wider oscillation of stapes --> increased sensitivity to sound
144
Prolonged exposure to loud noises causes loss of hearing; how?
Damage to stereociliated hair cells of organ of Corti
145
Damage to what structures causes conductive hearing loss?
Rupture of tympanic membrane
| Defects of middle ear ossicles
146
Pathway of sound transmission to organ of Corti
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
Carotid sinus baroreceptors & Aortic arch baroreceptors -- what innervates them? What occurs once receptors stimulated?
Carotid sinus - Glossopharyngeal n.
Aortic arch - Vagus n.
Stimulation of stretch receptors --> vasodilation, decrease in HR and contractility, decrease in BP
148
Spoon nails, dysphagia; what deficiency is present?
Iron deficiency anemia
esophageal web = Plummer-Vinson
microcytic hypochromic RBCs
rx: give iron preparation
149
Deficiency in vitamin B12 or folic acid -- what kind of anemia occurs?
Megaloblastic anemia
150
Deficiency in pyridoxine
Pyridoxine required as cofactor for 1st step in heme synthesis
Decreased heme synthesis --> microcytic, hypochromic pyridoxine-responsive anemia (sideroblastic anemia)
151
Alveolar cells containing golden cytoplasmic granules --> dark w/ Prussian blue staining; what is pathology?
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
Turner's syndrome: levels of FSH/LH?
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
Fibroadenoma
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
Ductal carcinoma in situ (DCIS)
precancerous breast lesion
malignant clonal cell proliferation (contained by surrounding ductal BM)
Myopethelial (basal) layer preserved/uninvolved
155
Paget disease of nipple
Malignant cells spread from superficial DCIS into nipple skin (does not cross BM)
unilateral erythema and scale crust around nipple
156
Sclerosing adenosis
Central acinar compression/distortion (by surrounding fibrotic tissue) and peripheral ductal dilation
common finding in fibrocystic change
157
Mammary duct ectasia
Ductal dilation, inspissated breast secretions
Chronic granulomatous inflammation in periductal and interstitial areas
158
Medullary carcinoma
Solid sheets of vesicular, pleomorphic, mitotically active cells w/ significant lymphoplasmacytic infiltrate around/within tumor -- pushing, noninfiltrating border
159
Temporal arteritis
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
Reversal treatment for ingestion of rat poison
Pts. susceptible to GI bleeding (no coagulation factors)
Give fresh frozen plasma and vitamin K1
161
Hamartoma
Excessive growth of tissue type native to organ of involvement
162
Most common benign lung tumor
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
Type of lung cancer w/ neuroendocrine markers
Small cell carcinoma of the lung
164
Keratin pearls characteristic of what type of malignancy?
Squamous cell carcinoma
165
Membranous GN
Nephrotic
Generalized edema
Heavy proteinuria (>3.5g/day)
Hypoalbuminemia
Hyperlipidemia
166
IgA nephropathy (Berger disease)
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
Squamous cell carcinomas of bladder
RARE
| Associated w/ Schistosoma haematobium infection
168
Urothelial carcinoma
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
Vitelline duct connects what two structures; when it it obliterated? What condition results if duct persists?
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
Umbilical hernia
Weakness of abdominal wall at umbilicus - midline protrusions covered by skin
171
Omphalocele
Midline defect in abdominal wall - incomplete closure -- abdominal organs protrude through defect covered w/ peritoneal sac
172
Facor XIII deficiency
clot instability --> delayed, recurrent bleeding after trauma
173
Protein C acts w/ Protein S to inactivate what factors?
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
Factor X activates thrombin; what does thrombin activate?
Thrombin activates factor V, VIII, and XI
175
ALS is combined UMN and LMN lesions: what structures involved?
Loss of neurons in anterior horn (LMN) - muscle weakness & atrophy
Degeneration/atrophy of lateral corticospinal tracts (UMN) - spasticity & hyperreflexia
176
Treatment of ALS
Riluzole
| MOA: decreases glutamate release
177
What structures are damaged in poliomyelitis?
Enteroviral infection damages motor neurons in anterior horn --> LMN disease
Flaccid paralysis, atrophy, areflexia, fasciculation
178
Huntington disease; what structures are damaged?
Caudate nucleus + putamen
179
Symptoms of Friedrich ataxia
```
AR disorder -- children 5-15yrs old
Ataxia
hypertrophic cardiomyopathy
kyphoscoliosis
foot abdnormalities
diabetes mellitus
```
180
Pathogenesis of Vitamin B12
Subacute combined degeneration of:
| posterior & lateral spinal columns
181
Tumor staging vs. grading
Stage - TNM system (tumor size, nodal involvement, metastasis)
Grade - differentiation (high = high cellularity, nuclear peomorphism, poorly differentiated)
182
Tumor with high bromodeoxyuridine uptake; what does this indicate?
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
Older female, weight loss, abdominal discomfort, jaundice, epigastric mass; what GI association?
Pancreatic cancer
184
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?
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
Early diastolic murmur heard better with handgrip exercise?
Aortic regurgitation
186
Mid-systolic pulmonary ejection murmur
due to increased flow across pulmonic valve
187
Incomplete obliteration of processus vaginalis = connection btwn peritoneum & scrotum; what are 2 conditions that can occur from this connection?
Hydrocele (fluid leakage)
| Indirect inguinal hernia (abdominal contents herniate down)
188
Enzyme missing in albinism
Tyrosinase (uses tyrosine as substrate for melanin)
189
Enzyme missing in alkatonuria
Homogentisic acid oxidase
Accumulation of homogentisic acid in body that needs to be renally excreted
190
Gene mutations associated w/ Alzheimer's
APP (chromosome 21)
presenilin 1 & 2
apoE4
APP & presenilin mutations promote AB-amyloid formation
191
Familial hypercholesterolemia
AD disorder
LDL receptor mutations -- liver can't take up LDL --> severe elevation in total cholesterol
192
Hypertrophic cardiomyopathy mutation
AD trait
mutation in B-myosin heavy chain
193
Renal artery stenosis often caused by atherosclerosis; what cells undergo hyperplasia/hypertrophy? Why?
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
Damage to common peroneal n. -- what structure is involved?
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
Deep peroneal n. (action)
Sensory to region btwn 1st and 2nd digits
| Anterior compartment of leg (dorsiflexors of foot and toes) -- in conjuction w/ common peroneal n.
196
Tibial n. (action)
Plantar flexion & inversion of foot
197
Important structures coursing near medial malleolus
Anteriorly:
Saphenous n.
Great saphenous vein
Posteriorly:
Posterior tibial a.
Tibial n.
Tendons of:
flexor digitorum longus
flexor hallucis longus
tibialis posterior
198
What type of gating does CFTR gene have?
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
Where does cGMP-gated Na+ channels play a role?
Vision
200
Persistent allantois remnant; what clinical features present?
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
Physical manifestations of 47, XXX
Silent - normal females
| some slightly decreased IQ scores
202
Physical manifestations of 47, XXY
Tall male
gynecomastia, small testes, infertility
Klinefelter syndrome
203
Physical manifestations of 47, XYY
Tall, severe acne
| Delay in motor & language development
204
Calcitriol
Active form of vitamin D converted in the KIDNEYS from 25-hydroxycholecalciferol
205
Actions of calcitriol
Increase Ca2+ & phosphate absorption
206
Chronic renal failure: what happens to PTH, Ca2+, phosphate, calcitriol?
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
Why does Ca2+ levels decrease in chronic renal failure?
Decrease excretion of phosphate --> phosphate binds Ca2+ to form complexes --> less free Ca2+
208
What two values always counteract each other in renal disease?
PTH and phosphate
High phosphate (lack of renal excretion) --> low Ca2+ --> elevate PTH
209
Conn's syndrome
primary hyperaldosteronism (zona glomerulosa hyperplasia)
primary adrenal hyperplasia
renal Na+ retention
excess K+ secretion
results in HTN and hypokalemia
Hypokalemia (muscle weakness); parethesias
210
Weight gain + easy bruising; what adrenal condition?
Cushing's syndrome
Glucocorticoid excess
Inhibited collagen and matrix GAG synthesis --> weaker skin/CT --> easy bruising
Zona fasiculata
211
Hirsutism (excessive hair growth); what adrenal condition?
Androgen excess - adrenal tumor causing androgen hypersecretion
zona reticularis
212
Diarrhea & flushing; what condition association?
Carcinoid syndrome
Serotonin(5-HT)-secreting tumor derived from neuroendocrine cells of GI tract
213
Chronic myeloproliferative disorders (4)
CML - leukocytosis w/ marked left shift
Essential thrombocytosis - thrombocytosis
Polycythemia vera - erythrocytosis
Primary myelofibrosis - bone marrow fibrosis
214
Mutation in myeloproliferative disorders (except for CML):
| primary myelofibrosis, essential thrombocytois, essential thrombocytosis
JAK2 - tyrosine kinase (phosphorylation)
JAK2 causes persistent activation of STAT (signal transducers & activator of transcription)
215
Primary myelofibrosis
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
Why is there hepatomegaly and splenomegaly in myelofibrosis?
Loss of bone marrow hematopoiesis --> extramedullary hematopoiesis
217
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-ketoglutarate dehydrogenase
cofactors (5):
Thiamine (deficient in alcoholics)
Lipoic acid
CoA
FAD
NAD+
```
conversion from a-ketoglutarate to succinyl-CoA
218
Presence of unilateral fetal hydronephrosis --> what structure is possibly affected?
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
Origin of ureteric bud?
Metanephros
220
Origin of collecting ducts, calices, renal pelvis, ureters, renal parenchyma?
Metanephros
221
Most common cause of bacterial meningitis in adults of all ages?
Streptococcus pneumoniae
Lancet-shaped, Gram+ cocci in pairs
Increase neutrophils, decrease glucose, increase opening pressure
Pt. w/ confusion, severe headache, nausea, nuchal rigidity
222
Pt. with Parkinsons complains of "freezing up" moments followed by episodes of not experiencing any rigidity; why?
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
What kind of pathology is associated w/ elevated conjugated bilirubin?
Hepatobiliary disease (cirrhosis or hepatitis) -- bilirubin conjugates will reflux back to plasma when secretion of bilirubin into bile is slow
224
What kind of pathology is associated w/ elevated Unconjugated bilirubin?
Increased bilirubin formation (hemolysis)
| Slowing in bilirubin conjugation (Gilbert syndrome)
225
Gilbert syndrome
UDP glucuronyl transferases decreased
Bilirubin glucoronidation decreased
226
What is the association btwn Wilson's disease and the liver?
Pt. younger than 30 yrs w/ unexplained chronic hepatitis (elevated AST and ALT)
Presence of ceruplasmin & urinary copper excretion
Kayser-Fleischer rings
227
Superior mesenteric artery syndrome; what are predisposing factors? At what level does SMA leave aorta?
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
What malignancies is Down syndrome associated w/?
AML
ALL
early Alzheimer
229
Cause of Cushing's syndrome: ACTH dependent vs ACTH independent
ACTH dependent:
a) Cushing's disease - pituitary ACTH hypersecretion
b) Ectopic ACTH syndrome - paraneoplastic ACTH secretion
ACTH independent:
a) adrenal adenoma or carcinoma
230
Embryonic aortic arch that ductus arteriosus derives from
6th
231
Patent ductus arteriosus; what to keep it open; what to close it?
```
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
How is gastric acid secretion by parietal cells in mucosal glands (fundus & body of stomach) stimulated?
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
What action does increased intracellular Ca2+ and cAMP have on gastric acid secretion?
Ca2+ increase (activates things)
cAMP activate protein kinases
BOTH lead to increased transport of H+ by H+K+ ATPase into gastric lumen
234
Systemic mastocytosis (symptoms)
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
Pernicious anemia
Autoantibody to parietal cells/intrinsic factor
IF not produced --> vit. B12 not absorbed --> neurologic symptoms
236
Substances that pancreatic tumors secrete:
1) Gastrin: ZE syndrome
2) Insulin: fasting hypoglycemia
3) Glucagon: diabetes mellitus & RASH*
4) Somatostatin: diarrhea, cholelithiasis, diabetes mellitus
237
Opening snap (extra heart sound) shortly after S2 heart sound (AV close); Diastolic rumbling murmur after opening snap best heard at heart apex
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
Congenital torticollis
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
What can bind to antithrombin? What does antithrombin do?
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
Enoxaparin
LMWH
241
Fondaparinux
Synthetic pentasaccharide factor Xa inhibitor
242
Relationship of ureters w/ common/external iliac vessels and gonadal vessels?
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
Ventromedial nucleus of hypotahalmus destroyed
Person eats more and becomes obese
| ventromedial nucleus responsible for satiety
244
Lateral nucleus of hypothalamus destroyed
Person starves (lateral nuclei responsible for hunger)
245
Supraoptic and paraventricular nuclei destroyed
No release of ADH or oxytocin (later carried down to neurohypophysis)
246
Anterior hypothalamic nuclei vs. posterior hypothalamic nuclei in relation to heat production
Anterior hypothalamic nuclei = vasodilation & sweating = COOL
Posterior hypothalamic nuclei = conserve heat, heat production = HOT
247
What morphological change occurs in vessels in primary pulmonary HTN? What causes it?
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
Rx for primary pulmonary HTN
Bosentan - endothelin-receptor antagonist
(endothelin vasoconstricts and stimulates endothelial proliferation) -- blocking of endothelin --> decrease pulmonary arterial pressure (lessen RV hypertrophy)
249
Pt. w/ chronic aortic stenosis (AS) experiences a. fib; what symptoms exacerbated? What could happen to venous pressures?
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
Hyperpyrexia (temp?); what should be done first?
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
Bacterial LPS can stimulate high fevers; what substances released to induce fever?
IL-1
IL-6
TNF
252
What vessel is derived from the common cardinal veins?
SVC
253
How does left heart failure lead to increased pulmonary arterial pressure?
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
Indirect vs. Direct vs. Femoral hernias
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
Hesselbach's triangle
Inguinal ligament - inferiorly
Inferior epigastric vessels - laterally
rectus abdominus - medially
Transversalis fascia - floor of triangle
256
Uncinate process of pancrease/major pancreatic duct - derived from?
Ventral bud
257
Pancreas divisum
Dorsal & ventral buds don't fuse
Clinically silent usually
Can predispose to recurrent pancreatitis; accessory duct drains majority of pancreas
258
Middle meningeal a. vs. middle cerebral 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
What does a decrease in CO in CHF patients do to renin-angiotensin system?
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
Where is angiotensinogen converted to angiotensin I?
Liver
261
Systolic dysfunction
Decrease in myocardial contractility
262
Diastolic dysfunction
Inability of heart to relax sufficiently to allow adequate filling of blood
263
Zollinger-Ellison; why is it associated w/ diarrhea?
Hypersecretion of gastrin via pancreatic tumor
Peptic ulcers result
Diarrhea -- due to inactivation of intestinal & pancreatic enzymes by EXCESSIVE GASTRIC ACID
264
Polyarteritis nodosa (PAN)
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
Buerger's disease (thromboangiitis obliterans)
Thrombosing vasculitis - medium/small sized arteries
Tibial and radial arteries
Heavy smokers, onset prior to 35
266
Takayasu arteritis
Giant cell (temporal) arteritis
Jaw claudication
Affects aortic arch
Females <40yrs
267
Lesions present in primary vs. secondary vs. tertiary syphilis
Primary - painless ulceration: chancre
Secondary - Condyloma lata: gray wart-like growths
Tertiary - Gummas: painless, indurated granulomatous lesions
268
Positive VDRL and pleocytosis in CSF: diagnosis?
Tertiary syphilis (neurosyphilis)
269
Condylomata acuminata
HPV 6 and 11
270
Sudden, painless, permanent monocular blindenss; pale retina & cherry red macula
Central retinal artery occlusion
271
What major arteries does the central retinal artery branch off from?
Ophthalmic artery (branch from internal carotid artery)
Embolism (from a. fib or carotid artery stenosis) --> central artery occlusion
272
Blurry vision, poor night vision, floaters, decreased peripheral vision; "cotton wool" exudates, "dot and blot" hemorrhages & new retinal vessel formation
Diabetic retinopathy
273
Amaurosis fugax
Painless, transient, monocular vision loss due to small embolus to ophthalmic artery
274
Temporal lobe infarcts - what vision changes occur?
Meyer's loop affected --> CONTRA upper quadrantanopsia
275
Large pituitary adenoma - what vision changes occur?
Bitemporal hemianopia
276
Fall on outstretched, dorsiflexed hand; what wrist bone is affected?
Lunate dislocation
Lunate next to the scaphoid bone -- both articulate w/ the radius --> avascular necrosis
277
Relative polycythemia
Dehydration
Excessive diuresis
Decreased plasma volume
Normal RBC mass
Normal SaO2 & EPO level
278
Primary polycythemia
Polycythemia vera
Increased plasma volume
Increased RBC mass
Decreased EPO**
279
Secondary polycythemia
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
Crohn's disease - association with kidney stones?
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
Lung physiology in patient w/ COPD
Increase TLC
Decrease FEV1/FVC
Decrease FVC
Increase RV
282
Why is there increase in both TLC and RV in COPD?
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
Lung physiology in patient with restrictive lung disease
Decrease TLC
Decrease FVC
Decrease RV
Increase FEV1/FVC
Increase elastic recoil
Decrease lung expansion
284
Ligation of the superior thyroid artery; what muscle may become affected if the nerve near the superior thyroid artery is damaged?
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
Paroxysmal breathlessness & wheezing; granule-containing cells with crystalloid bodies; what condition does the pt. have?
Allergic asthma
Granule containing cells = eosinophils
Crystalloid bodies = Charcot-Leyden crystals (contain eosinophil membrane protein)
286
What factor activates eosinophils in pts. w/ chronic eosinophilic bronchitis?
IL-5 released by TH2 cells
287
Differential diagnosis for decreased visual acuity & tunnel vision in low light (night blindness)
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
Crescendo-Decrescendo systolic murmur: what valve disease?
Aortic stenosis
Due to:
Degenerative (senile) calcinosis of aortic leaflets
289
Mitral stenosis;
| Mitral & aortic regurgitation -- what disease association?
Rheumatic fever
290
Cystic medial necrosis - what predisposing factors?
Cystic medial necrosis --> aortic dissection (abrupt severe chest pain) --> dilation of aortic root (aortic regurgitation)
291
Broca's aphasia; what area of brain affected?
Inferior frontal gyrus of dominant (usually left) hemisphere
292
Precentral vs. Postcentral gyrus damage: effects?
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
What nerve innervates muscles of mastication?
CNV3 (mandibular branch) - exits via foramen ovale
Masseter, medial pterygoid, temporalis m. = close jaw
Lateral pterygoid = opens jaw
294
Child w/ white pupillary reflex; what malignancy? What associated conditions?
Retinoblastoma due to Rb gene mutation
Familial retinoblastoma --> osteosarcoma later in life
Rb gene mutations - usually associated w/ 2-Hit hypothesis
295
Ewing sarcoma
Neoplasm of neuroectodermal cells
Long and flat bones of children/adolescents
296
Most common malignant brain tumor of childhood
Medulloblastoma (arises in cerebellum)
297
What causes proteolysis of factor V and VIII?
Protein C (actions of protein C increased by protein S)
Protein C and Protein S prevent thrombosis
298
Radial nerve deficit; what symptoms? What fracture results in radial n. deficit?
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
Damage to what nerve affects opposition of thumb?
Median n.
