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Pathology
Pathoma pathology high-yield (chapters 5-19)
816
Pathology
Graduate
04/15/2013

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Term
What is the classic stain for Acute myeloid leukemia?
Definition
Positive for MPO therefore will see Aeur rods
Term
Which AML subtype is associated w/ Down's syndrome before the age of 5?
Definition
M7
Term
Which AML subtype classically presents w/ gum infiltration?
Definition
M5: acute monocytic leukemia
Term
Which AML subtype has the t(15,17)? What is the treatment?
Definition
M3, Tx w/ ATRA (all trans-retinoic acid receptor)
Term
What is the classic nuclear staining for Acute lymphoblastic leukemia that will distinguish it from AML?
Definition
Tdt = DNA polyermase; present ONLY in the nucleus of T and B cells
Term
Which ALL subtype is more common? B or T cells? And what are the CD markers that are used to distinguish them?
Definition
B-ALL. CD10+, CD19+, CD20+
Term
In B-ALL which translocations has a good prognosis and which has a bad one?
Definition
t(12; 21): good; seen in kids. T(9;22): poor; seen in adults- this is classicfied as Ph+ ALL
Term
When does T-ALL present? How does it present?
Definition
It presents in teenagers as a mediastinal (thymic) mass; called acute lymphoblatic lymphoma b/c the malignant cells form a mass
Term
What is the CD marker for Chronic Lymphocytic Leukemia (CLL)?
Definition
CD5+CD20+; proliferation of anive B cells
Term
What type of Leukemia and lymphoma do you see a "smudge" cell
Definition
CLL and SLL (small lymhocytic lymphoma)
Term
What is the most common cause of death in patients w/ CLL and why?
Definition
Infection due to Hypogammaglobulinemia
Term
what is the type of cell that undergoes neoplastic proliferation in Hairy cell Leukemia?
Definition
Mature B cells
Term
What does it mean if a cell is TRAP positive (in the context of leukemias)?
Definition
That they're a Hairy Cell Leukemia
Term
When would you find a "dry tap" in leukemia and what does this mean?
Definition
In Hairy Cell Leukemia, this means that the BM is dry dry due to marrow fibrosis
Term
What is a clinical feature of Hairy Cell leukemia?
Definition
Splenomegaly
Term
Which part of the spleen gets trapped w/ TRAP + cells in Hairy Cell Leukemia that leads to splenomegaly?
Definition
The RED PULP!
Term
What drug is the treatment for Hairy Cell Leukemia?
Definition
2-CDA (cladribine): adenose deaminase inhibitor- adenose accumulates to toxic level sin neoplastic B cells
Term
What virus can lead to Adult T-Cell Leukemia/lymphomas? What parts of the world is this virus seen in ?
Definition
HTVL-1; seen in Japan and the Carribean?
Term
What do you suspect if the patient has a rash AND lytic (punched-out) bones lesions w/ hypercalcemia?
Definition
Adult T-cell Leukemia/lymphomas
Term
What do you suspect if the patient has lytic (punched-out) bones lesions w/ hypercalcemia but NO RASH?
Definition
Multiple Myeloma
Term
What leukemias do you suspect if the patient has a rash?
Definition
Mycosis fungoides and TLLL
Term
What is a Pautrier microabscess? When do you see this?
Definition
Aggregrates of neopalstic cells in the epidermis but NOT a true microabscess since there are no neutrophils; seen in Mycoses fungoides
Term
When does Sezary syndrome occur? What does the characteristic cell look like?
Definition
It occurs in mycosis fungoides when the cels have spread to involve the blood; the Sezary cells has a cerebriform nucleus.
Term
What does it mean to have a t(9;22) marker?
Definition
Philadelphia chromosome that generates a BRC-ABL fusion --> increased tyrosine kinase activity which is a signal transducer?
Term
Which diseases are t(9;22) positive?
Definition
CML and Ph+ ALL
Term
If a patient w/ CML has splenomegaly what can occur next?
Definition
It can transform into either AML (2/3 of cases) or ALL (1/3 of cases)
Term
How do you distinguish CML from a leukemoid rxn?
Definition
Neg LAP stain, increased basophils, t(9;22) positive
Term
What mutation is Polycythemia vera associated with?
Definition
JAK2 kinase mutation
Term
What is the most common venous thrombosis that can arise in patients w/ Polycythemia vera?
Definition
Hepatic vein thrombosis leading to Budd Chiari syndrome
Term
What is the treatment for CML?
Definition
Imatinib: blocks tyrosine kinase activity
Term
Why do Polycythemia vera patients get itchy after bathing?
Definition
Due to histamine release from increased maset cells
Term
How do you distinguish Polycythemia vera from reactive polycythemia?
Definition
in PV, EPO is decreased, SaO2 is normal. In reactive poly due to high altitude or lung diseae: SaO2 is low and EPO is increased.
Term
What tumor produces EPO leading to polycythemia vera?
Definition
Renal cell carcinoma
Term
what are the clinical feature of myelofibrosis?
Definition
Splenomegaly due to extramedullary hematopoiesis, leukoerythroblatic smear (tear drop cells), and increased risk of infection/thrombosis/bleeding
Term
what 3 chroninc myeloproliferative disorders are associated w/ JAK2 mutations?
Definition
Polycythemia vera (100%), essential thrombocytosis (30-50%), melofibrosis (30-50%)
Term
In EBV, which part of the lymph node and which part of the spleen undergo hyperplasia?
Definition
LN: paracortex; Spleen: PALS b/c these are where the T cells live.
Term
When would you see follicular hyperplasia in the lymph node?
Definition
Rheumatoid arthrisis and early stages of HIV infection
Term
What disease is associated w/ the translocation t(14;18)?
Definition
Follicular cell lymphoma
Term
What does the translocation t(14;18) encode for?
Definition
Bcl-2 on Ch 18 translocates to the Ig Heavy chain locus on Ch 14 --> overexpression of Bcl2 --> INHIBITS apoptosis
Term
What is the treatment for Follicular cell lymphoma?
Definition
Rituximab
Term
How do you distinguish Follicular cell lymphoma from follicular hyperplasia?
Definition
1. Disruption of normal architecture, 2. Lack of tingible body macrophages in germinal centers since no apoptosis is occuring, 3. Bcl2 expression in follicles (so NO apoptosis is occuring), 4. Monoclonality whereas follicular hyperplasia is polyclonal.
Term
What type of lymphoma is described as "a tumor that expands the region immediately adjacent to the follicle" in a lymph node?
Definition
Mantle cell lymphoma
Term
Which lymphoma has the translocation t(11:14)? What does this translocation mean?
Definition
Mantle cell lymphoma; Cyclin D1 on Ch11 is translocated to the Ig heavy chain on Ch 14 --> overexpression of Cyclin D1 which promotes G1/S transition in the cell cycle --> cell growth
Term
What type of lymphoma is classically associated w/ chronic inflammatory states like: Hashimotos thyroiditis, Sjogren syndrome and H. pylori gastritis?
Definition
Marginal zone lymphoma
Term
Where does the African form of Burkitt's lymphoma arise? What about the Sporadic form?
Definition
African: jaw; Sporadic: abdomen
Term
What drives cell growth in Burkitt's lymphoma? What's the translocation for it?
Definition
Overexpression of c-myc b/c of the t(8;14) translocation which oplaces the c-myc from Ch 8 onto the Ig heavy chain of Ch 14.
Term
When do you get a "starry-sky appearance" on histology?
Definition
Burkitt's lymphoma
Term
What is the most common form of Non-Hodgkin's lymphoma?
Definition
Diffuse B-large cell lymphoma
Term
What is a cell w/ a "owl-eye nuclei" that is also CD15+CD30+?
Definition
Reed Sternberg cell seen in Hodgkin lymphoma
Term
What is the most common form of Non-Hodgkin's lymphoma?
Definition
Nodular sclerosis; typically seen in a young adult female
Term
What is a Lacuna cell in a NHL?
Definition
It's a Reed Sternberg cell that is seen in nodular sclerosis.
Term
Which has the best best prognosis? Lymphocyte-rich or lymphocyte-poor Hodgkin lymphoma?
Definition
Lymphocyte-rich: best prognosis
Term
What receptor does the neoplastic cell in multiple myeloma activate on osteoclasts? What does this lead to?
Definition
It activates RANK --> leads to bone destruction --> lytic "punched out" lesions on X-ray
Term
What type of protein is excreted in the urine in patients with Multiple myeloma?
Definition
Bence Jones proteins (free light chain)
Term
What is the Ig spike in patients w/ Multiple myeloma?
Definition
IgG (55%) or IgA (25%)
Term
What are the 3 fates of the free light chain that is produced in multiple myeloma?
Definition
1. Deposition into the blood and tissue leading to Primary AL amyloidoisos, 2. Proteinuria and 3. epsition into the kidney tubules --> renal faiulre (myeloma kidney)
Term
What is MGUS?
Definition
Monoclonal gammapathy of undeterminde significance w/o the clinical features of multiple myeloma
Term
In which typ eof disease do you see Birbeck (tennis racket) granules on electron microscopy? What are they? What are the CD markers assocaited with them?
Definition
Seen in Langerhans cell histiocytosis (Letter-Siwe disease, Eosinophilc granuloma, and Hand-Schuller-Christian disease); they're langerhans cells; they're CD1a+ and S100+
Term
What type of cell in Multiple myeloma has a "fried egg" appearance to it?
Definition
A plasma cell (Ab producing cell)
Term
Which part of the blood vessel is affected in atherosclerosis?
Definition
The intima of arteries in either medium or large blood vessels. there is an intima plaque w/ a chronic lipid core and a fibromuscular cap; often undergoes DYSTROPHIC calcification.
Term
Which blood vessels are the most commonly involved in atherosclerosis?
Definition
The abdominal aorta, the coronary artery, the popliteal artery and the internal carotid artery
Term
What are the 4 modifiable risk factors in atherosclerosis?
Definition
HTN, smoking, diabetes and hypercholesterolemia
Term
What is the % occlusion at which symptoms occur in atherosclerosis?
Definition
>70%
Term
What is the hallmark of an artherosclerotic emboli?
Definition
Cholesterol clefts (crysals) w/in the embolus
Term
What are the TWO reasons that patiens develop hyaline arteriolosclerosis? THIS IS HIGH YIELD
Definition
1. Benign HTN and 2. Diabetes
Term
What is hyaline arterioscllerosis?
Definition
It is caused by proteins being leaked into the vessel wall leading to the thickening of the vessel wall. 1. Benign HTN: the increased BP forces proteins into the wall. 2. Diabetes: non-enzymatic glycosylation of basement membrane --> makes Blood vessel leaky allowing for proteins to get in.
Term
What type of arteriolosclerosis does malignant HTN result in? What does it look like?
Definition
Hyperplastic arteriolosclerosis (thickening of the blood vessel wall due to hyperplasia of smooth muscle) that looks like "onion-skin" appearance.
Term
What can hyperplastic arteriolosclerosis lead to?
Definition
Fibrinoid necrosis --> may cause acute renal failure
Term
If the kidney has a "flea bitten" appearance to it, what does that indicate?
Definition
Renal failure due to hyperplastic arteriolosclerosis due to malignant HTN
Term
What is the major complication of a thoracic aneurysm?
Definition
Dilatation of the aortic valve root --> resulting in aortic valve insufficiency
Term
What is a "tree bark' appearance of the aorta due to?
Definition
Scarring and fibrosis b/c of tertiary syphilis
Term
What two syndromes result in weakness of the connective tissue in the media (cystic medial necrosis) that can lead to an aortic dissection?
Definition
Marfan syndrome and Ehlers-Danlos syndrome
Term
What two things are required to occur to mediate an aortic dissection?
Definition
High stress and pre-existing weakness of the media
Term
where does an abdominal aortic aneursym occurs?
Definition
Below the renal arteries and above the aortic bifurcation
Term
What is the dilation of the AAA that is most likely to rupture?
Definition
>5cm
Term
What part of the aorta does a Type A aortic dissection involve? What is the management that a patient needs that presents w/ a Type A dissection?
Definition
The ascending and descending aorta; need surgical management immediately
Term
What part of the aorta does a Type B aortic dissection involve? What is the management that a patient needs that presents w/ a Type B dissection?
Definition
The descending aorta. Needs Medical management; beta blocker is the drug of choice. It decreases the slope of the rise of the BP which slows down the progression of the dissection.
Term
What is the classic finding on EKG for stable angina? What is the tx for angina?
Definition
ST segment depression. Tx: rest and nitroglycerin
Term
What is the classic finding on EKG for Prinzemetal angina? Treatment?
Definition
ST segment elevation due to transmural ischemia. Tx: nitro or CCB
Term
What cardiac marker is useful for detecting reinfarction on top of an acute MI?
Definition
CK-MB
Term
What cardiac marker is the good standard for detecting an MI?
Definition
Troponin I
Term
In the first 6hrs of an MI, what should you do in terms of testing?
Definition
EKG is gold standard
Term
What are some of the MOST important complications that can stem from an MI one week post an MI?
Definition
Rupture of ventricular free wall (leads to cardiac tamponade), rupture of IV septum (leads to shunt being created), and Rupture of papillary muscle which is fed by the RCA that can lead to mitral insufficiency.
Term
What is Dressler syndrome?
Definition
It is an autoimmune phenomenon resulting in fibrinous pericarditis (several weeks post MI). Basically the pericardium is exposed and the body decides to make antibodies against it.
Term
What occurs 4-24hrs after an MI?
Definition
The heart looks dark in appearance where the MI occurs b/c of coagulation necrosis. The myocytes have lost their nuclei.
Term
With what type of heart failure do you see "heart-failure" cells in the lungs? What are these cells?
Definition
You see it w/ Left sided heart failure. These are hemosiderin-laden macrophages.
Term
What is the most common cause of Right sided heart failure?
Definition
Left sided heart failure
Term
What are some clinical features of RHF?
Definition
Jugular venous distension. Painful hepatosplenomegaly w/ characteristic nutmeg liver; pitting edema (due to increased hydrostatic force)
Term
What are the 3 Left to right shunts?
Definition
VSD, PDA, ASD
Term
What is the most common congenital cardiac anomaly? What syndrome is it assocaited with?
Definition
VSD; associated w/ Fetal alcohol syndrome
Term
How do L to R shunts become R to L shunts?
Definition
Eisenmenger's syndrome. Uncorrected VSD, ASD, or PDA --> compensatory pulmonary vascular hypertrophy --> results in progresive pulmonary HTN --> as the pulmonary resistannce Increases, the shunt reverses from L to R to R to L. This causes late cyanosis w/ clubbing of the fingers and polycythemia.
Term
What type of murmur causes a loud S1 and wide, fixed split S2?
Definition
ASD
Term
What is an ASD? What's the cuase? What's the most common type in patients w/ Down syndrome?
Definition
It is an atrial septal defect; failure of either the ostium primum or secundum to occur. Osteium secundum is the most common type. Ostium primum is the most common type in DS kids.
Term
What is the most common congenital cardiac defect assocaited w/ congenital rubella?
Definition
PDA
Term
What is a PDA?
Definition
It is a Left to Right shunt b/w the aorta and the pulmonary artery.
Term
What type of murmur sounds like a holosystolic "machine-like" murmur?
Definition
PDA
Term
What drug is used to close a PDA?
Definition
Indomethicin b/c it decreases PGE
Term
If a child (not a baby) presents w/ a holosystolic "machine-like" murmur and lower extremity cyanosis, what happened to this kid?
Definition
He has a PDA at birth then it reversed and lead to Eisenmerger syndrome.
Term
What are the components of tetralogy of fallot?
Definition
P: pulmonary stenosis. R: RVH, O: Over-riding aorta (it overrisdes the VSD), V: VSD
Term
What type of shunt does Tetralogy of fallot result in?
Definition
Right to left shunt
Term
What type of congenital cardiac defect is associated w/ a "boot shaped" heart on X-ray?
Definition
Tetralogy of fallot
Term
What type of congenital cardiac defect is associated with maternal diabetes?
Definition
Transposition of the great vessels
Term
Name the Right to Left shunts that result in "blue babies"
Definition
Tetralogy of fallot, Transposition of the Great Vessels, Truncus arteriosus, Tricuspid atresia, Total anomalous pulmonary venous return.
Term
What leads to transposition of the great vessels?
Definition
The aorta comes off of the RV (instead of coming off of the LV) and the pulmonary artery comes off of the LV (instead of coming off of the RV) which is going to create two separate circulations.
Term
What other shunt is transposition of the great vessels associated with? Do we want to keep that open? How do you keep the shunt open?
Definition
Is it associated w/ PDA. We want to keep it open until the transposition of the great vessels can be corrected. We keep it open w/ PGE
Term
If an infant that is diagnosed w/ Turner syndrome presents w/ lower extremity cyanosis, what type of congential cardiac defect do you think the infant has?
Definition
Coarctation of the aorta: infantile form.
Term
If an adult presnts w/ HTN in the upper extremities and hypotension and weak pulses in the lower extremities,what type of heart issue does he have?
Definition
Coarction of the aorta: adult form.
Term
What key X-ray finding will you see in an adult w/ Coarction of the aorta: adult form?
Definition
"Notching" of the ribs
Term
What type of weird vavle is the adult form of Coarction of the aorta associated with?
Definition
Bicuspid aortic valve
Term
Where is the stenosis in the aorat in the infantile and the adult forms of coarctation of the aorta?
Definition
Infant: aortic stenosis proximal to insertion of ductus arteriousus (preductal). Adult: stenosis is distal to ligamentum arteriosum (post ductal)
Term
How do you distinguish aortic stenosis that occurs from natural "wear and tear" from that associated w/ chronic rheumatic fever?
Definition
Chronic rheumatic fever: there is always mitral stenosis present and there is always fusion of the aortic valve commissures present. These do NOT occur in aortic stenosis due to "wear and tear"
Term
What are the JONES criteris for Rheumatic fever?
Definition
Joints (migratory polyarthritis), Pancarditis, Subcutaneous nodules, Erythema marginatum, Sydenham chorea
Term
What are the cardiac issues associated w/ Rheumatic fever?
Definition
Endocarditis: mitral valve regurgitation occurs b/c of small vegetations that are present on the mitral valve. Aortic stenosis also occurs, but less frequently. Myocarditis: Ashcoff bodies (giant cells) w/ Anitschkow cells present inside of them (reactive histiocytes w/ slender, wavy nuclei that looks like a caterpillar). Pericarditis: friction rub and chest pain
Term
What are Anitschkow cells associated with?
Definition
Myocarditis due to Rheumatic fever
Term
What type of murmur am I? Systolic ejection click followed by a crescendo-decrescendo murmur?
Definition
Aortic stenosis
Term
What type of murmur am I? Early, high pitched blowing diastolic murmur
Definition
Aortic regurgitation
Term
If the patient presents w/ bounding pulse (water-hammer pulse), pulsating nail bed (Quincke pulse) and head bobbig w/ a wide pulse pressure, what type of murmur does he have?
Definition
Aortic regurgitation
Term
Why is there a wide pulse pressure in aortic regurgitation?
Definition
Diastolic pressure decreaes due to regurgitation. Systolic pressure increases due to increased stroke volume
Term
What type of murmur am I? High pitched holosystolic "blowing murmur" that gets louder w/ squatting and expiration?
Definition
Mitral regurgitation
Term
What type of murmurs are a) acute rheumatic fever and b) chronic rheumatic fever associated with?
Definition
a) Mitral regurgitation and b) Mitral stenosis
Term
What type of murmur am I? Opening snap followed by diastolic rumble
Definition
Mitral stenosis
Term
What type of murmur am I? Late systolic crescendon murmur w/ midsystolic click
Definition
Mitral valve prolpase
Term
What is the most common bug that causes endocarditis? Why does it cause subacute endocarditis?
Definition
Streptococcus viridians; low virulence and will only cause issues when there is pre-existing damage to the valves
Term
What is the most common cause of endocarditis in IV drug users? Which is the valve that it affects?
Definition
Staphylococcus aerues; Tricuspid valve. Don’t TRI drugs!
Term
What is the most common bug that is associated w/ endocarditis of prosthetic valves?
Definition
S. epidermidis (gram + cocci, catalase +, Novobiocin sensitive)
Term
What is the most common bug that is associated w/ endocarditis in patients w/ underlying colorectal carcinoma?
Definition
Strep bovis
Term
What organism should you suspect if the patient has endocarditis w/ negative blood cultures?
Definition
HACEK
Term
What are the clinical features of endocarditis?
Definition
FROM JANE. F: Fever (most common symptom), R: Roth's spot (retina and rare), O: Osler's nodes that are tender lesionson fingers/toes), M: Murmur (new mitral regurgitation), J: Janeyway lesions )painles erythematous lesions on palms/soles), A: Anemia, N: Nail-bed hemorrhages (splinter), E: Emboli
Term
What endocarditis is associated w/ SLE?
Definition
Libman-Sacks endocarditis
Term
What is a key finding of Libman-Sacks endocarditis (in terms of where the vegetations are present)?
Definition
Vegetations are present on BOTH the surface and undersurface of the mitral valve --> mitral regurgitation
Term
What are the findings for anemia of chronic disease?
Definition
Decrease Hb, Decrease MCV, Increase ferritin, Decrease TIBC, Decrease serum iron and Decrease % saturation
Term
What are the ABCCCD of dilated (congestive) cardiomyopathy?
Definition
A: alcoholic abuse, B: Beriberi (wet), C: coxsackie B virus (most common cause), C: Cocaine use, C: Chaga's disease, D: doxorubicin toxicity, hemochromatosis and peripartum cardiomyopathy )pregnancy associated)
Term
What heart sound will you hear in dilated cardiomyopathy? What type of apperance is seen on X-ray?
Definition
S3 heart sound; Balloon appearnce on X-ray
Term
What type of dysfunction occurs in dilated cardiomyopathy? Systolic of diastolic dysfunction?
Definition
Systolic dysfunction that will lead to both mitral and tricuspid valve regurgitations
Term
What is the most common cause of sudden death in young athletes?
Definition
Hypertrophic cardiomyopathy
Term
What is the classic finding on histology of hyptertrophic cardiomyopathy?
Definition
Myofiber hypertrophy w/ disarray; get concentric hypertrophy (sarcomeres added in parallel [disarray])
Term
What is the most common reason that a person gets Hypertrophic cardiomyopathy?
Definition
Autosomal dominant mutation in sarcomere proteins
Term
What dysfunction is seen in hypertrophic cardiomyopathy? Systolic or diastolic?
Definition
Diastolic dysfunction (ventricle cannot fill)
Term
What is the classic finding on EKG for restrictive cardiomyopathy?
Definition
Low voltage-EKG w/ diminished QRS amplitude?
Term
What are the causes of restrictive cardiomyopathy?
Definition
Amyloidosis, sarcoidosis, hemochromatosis, endocardial fibroelastosis (thick fibroelastic tissue in endocardiium of young kids), Loeffler's syndrome (endomyocardial fibrosis w/ an eosinophilic infiltrate aand eosinophili
Term
What is the most common cardiac tumor in adults? Why does it cause syncope?
Definition
Myxoma; causes syncope b/c it obstructs the mitral valve since it usually forms as a pedunculated mass in the left atrium
Term
What is the most common cardiac tumor in children? What is it associated with? Where does it arise?
Definition
Rhabdomyoma; associated w/ tuberous sclerosis. Usually arises int eh ventrilces
Term
If a child develops nasal polyps, which disease should he be tested for?
Definition
Cystic fibrosis
Term
What is the triad of aspirin-intolerant asthma?
Definition
Asthma, aspirin-induced bronchospasms and NASAL polyps!
Term
What population are angiofibromas classicaly seen? What is the classic presentation?
Definition
Adolescent males; profuse epistaxis
Term
A Chinese adult male presents w/ large cervical lymph nodes and the biopsy reveals pleomorphic keratin-positive epithelial cels in a background of lymphocytes. What is this? What is it associated with?
Definition
Nasopharynegeal carcinoma; associated w/ EBV
Term
Most common cause of acute epiglottitis?
Definition
H. influenzae type b
Term
Most common cause of laryngotracheobronchitis (CROUP)?
Definition
Parinfluenza virus (which is a paramyxovirus)
Term
What causes laryngeal papilloma?
Definition
HPV 6 and 11;
Term
What is the presentation of the laryngeal papilloma in adults vs kids?
Definition
Presents as a single papilloma in adults and as multiple in children
Term
What are the risk factors for laryngeal carcinoma?
Definition
Alcohol and tobacco; can rarely arise from a plaryngeal papilloma
Term
What are the two key mediators of pain?
Definition
Bradykinin and PGE2
Term
What are the two most common causes of lobar pneumonia?
Definition
Streptococcus pneumoniae and Klebsiella pneumoniae
Term
What bug causes lobar pneumonia in patients that are at increased risk for aspiration? What color is the sputum?
Definition
Klebsiella pneumoniae; Currant jelly sputum
Term
What is the stem cell of the lung that helps regenerate the lining of the lung?
Definition
Type II pneumocytes
Term
What are the gross phases of lobar pneumonia?
Definition
Congestion --> red hepatiation --> gray hepatization --> resolution
Term
What is the most common cause of secondary pneumonia (after a viral UTI)? What are the complications associated w/ this organism?
Definition
S. aureus; abscess or empyema
Term
What are the most common causes of broncopneumonia?
Definition
S. aureus, H. influenzae, P aeruginosa, Moraxella catarrhalis, Legionella pneumophila
Term
What are the most common causes of interstitial (atypical) pneumonia?
Definition
Mycoplasma pneumoniae, Chlamydia penumoniae, RSV, CMV, influenza virus, Coxiella burnetti
Term
What is the most common cause of interstitial (atypical) pneumonia? What is a very common complication?
Definition
Mycoplasma pneumoniae; autoimmune hemolytic anemia (IgM against I Ag on RBCs --> cold agglutinins)
Term
What is the most common cuase of interstitial (atypical) pneumonia in infants?
Definition
RSV
Term
What is the most common cuase of interstitial (atypical) pneumonia in patients w/ post transplant immunosuppressive therapy?
Definition
CMV
Term
What are the 3 most common bugs that cause aspiration pneumonia?
Definition
Bacteriodes, Fusobacterium and Peptococcus
Term
Which lobe does an asbcess occur in w/ aspiration pneumonia?
Definition
Right lower lobe
Term
What type of granuloma is seen in TB?
Definition
Caseating granulomas
Term
What is unique about the type of meningitis that is seen in TB patients?
Definition
Meningitis w/ granulomas at the base of the brain
Term
What is the most common tissue involved in systemic spread of TB?
Definition
Kidney --> sterile pyuria
Term
What are the key spirometry readings for chronic obstructive pulmonary disease?
Definition
Decrease FVC, Decrease FEV1, Decrease FEV1: FVC ration, Increased TLC
Term
What is the definiion of chronic bronchitis? What is it high associated with?
Definition
Chroninc productive chough lasting at least 3 months over a min of 2 years; highly associated w/ smoking.
Term
What is the Reid index? What is the value for chronic bronchitis?
Definition
It is the measure of the mucus glands relative to overall bronchi wall thickness. It's normally <40%, but it's >50 in chronic bronchitis.
Term
What type of cell undergoes hypertrophy in chronic bronchitis?
Definition
Bronchial mucinous glands
Term
What disease presents as "blue bloaters"? Why?
Definition
Chronic bronchitis; Mucus traps CO2 --> increase PaCO2 and Decrease PaO2
Term
What are some complications of Chronic bronchitis?
Definition
Increased risk of infection and cor pulmonale (failre of right heart b/c the lungs are vasoconstricted sincere there is Decrease PaO2)
Term
What are the underlying problems in emphysema?
Definition
Loss of elastic recoil and collapse of airyways during exhalation --> obstruction and air trapping. Due to either an overproduction of proteases or a decrease in antiproteases
Term
What is the most common cause of emphysema?
Definition
Smoking --> results in excess inflammation --> protease increases --> causes damage of the alveolar air sacs --> centriacinar emphysema most severe in the upper lobes
Term
What does alpha-1 antitrypsin (A1AT) deficiency result in?
Definition
Emphysema
Term
What type of emphysema will A1AT deficiency patients get?
Definition
Panacinar (panlobar) emphysema
Term
What is the most common clnically relevant mutation for A1AT deficiency?
Definition
PiZ: results in significantly low levels of circulating A1AT protein that will accumulate in the endoplasmic reticulum (PAS+)
Term
Who are "pink puffers" and why does this occur?
Definition
Emphysema patients; porlonged expireation w/ purpsed lips that creates back pressure that allows air to move in and out which allows for oxygenation to take place; but this requries lots of work and these patients are classically thin.
Term
What happens to the AP diameter of patients w/ emphysema?
Definition
It increases given them an appearance of a barrel chest
Term
Why does the AP diameter increase in patients w/ emphysema?
Definition
B/c the set point (FRC) increases
Term
What are the classic Th2 cytokines and what do they do?
Definition
IL-4: mediates class switch to IgE, IL-5: attracts eosinophils; IL-10: stimulates Th2 cells and inhibits Th1 cels
Term
What are the key findings of the sputum of asthmatics?
Definition
Curschman spirals and charcot-leyden cryals (eosinophil-derived crystals)
Term
Which part of the cilia is affected in Kartagener's syndrome?
Definition
The dynein arm
Term
What conditions can lead to bronchiectasis?
Definition
Cystic fibrosis, Kartagener syndrome, tumor or foreign body, necrotizing infection and allergic bronchopulmonary aspergillosis (ABPA)
Term
Does bronchiectasis lead to primary or secondary amyloidosis?
Definition
Secondary amyloidosis w/ deposition of AA.
Term
What are the key spirometry readings for restrictive diseases?
Definition
Decrease TLC, Decrease FVC, Increased to normal FEV1:FVC ratio (> 80%). These patients can't fill the lung vs COPD patients that can't expell air from the lungs.
Term
What is the key cytokine involved in Idiopathic pulmonary fibrosis (restrictive disease) that induces fibrosis?
Definition
TGF-beta
Term
In which disease will you see a "honeycomb" lung?
Definition
Idiopathic pulmonary fibrosis (restrictive disease)
Term
What is Caplan syndrome?
Definition
Rheumatoid arthritis that is associated w/ Coal worker's pneumoconiosis
Term
What is anthracosis?
Definition
Collections of carbon-laden macrophages
Term
What are the diffferent types of pneumoconioses (restrictive lung diseases)?
Definition
Coal Worker's pneumoconiosis, silicosis, berylliosis, asbestostis
Term
Which is the ONLY pneumoconioses that increases the risk for Tb?
Definition
Silicosis
Term
Differential Dx for: noncaseating granulomas in the lung, hilar lymphn nodes and systemic organs?
Definition
Sarcoidosis and berylliosis (pick this if it's beryllium miners and workers in the aerospace industry)
Term
What is a ferruginous body and where is it seen?
Definition
Long golden brown fibers w/ associated iron; confrms exposure to asbestos
Term
What is an asteroid body characteristic for?
Definition
Sarcoidosis
Term
In any disease that has noncaseating granulomas, why can these patients also have hypercalcemia?
Definition
The noncaseating granuloma contains 1-alpha-hydroxylase which converts Vitamin D to it's active form.
Term
What is the classic presenting sign of pulmonary HTN?
Definition
Exertional dyspnea
Term
What are plexiform lesions indicative of?
Definition
Long standing pulmonary HTN.
Term
What is the gene that is classically associated w/ familyial forms of primary pulmonary HTN? What does it do?
Definition
Inactivating mutation of BMPR2: leads to proliferation of vascular smooth muscle
Term
What is the hallmark of acute respiratory distress syndrome (ARDS)?
Definition
Hyaline membrane formation in the alveoli
Term
What is one of the key treatments for ARDS (besides treating the underlying cause)?
Definition
Ventilation w/ positive end-expiratory pressure (PEEP)
Term
What is the key immune cell that is involved in ARDS and what does it do?
Definition
Activation of neutrophils induces protease-mediated and free radial damage of type I and II pneumocytes.
Term
What could be a complication of recovery of ARDS and why does this occur?
Definition
Interstitial fibrosis b/c of damage/loss of type II penumoclyes --> scarring and fibrosis. You can't regenerate but you can repair the lung.
Term
What is the main component of surfactant?
Definition
Phosphatidlcholine (which is the same as Lecithin)
Term
What happens to the Lecithin: sphingomyelin ratio as the lungs mature?
Definition
Lecithin increases (since more surfactant is being produced) and sphingomyelin remains constant. The L:S ratio increases. A ratio of >2 = adequate surfactant production.
Term
Why does maternal diabetes increase the risk of neonatal respiratory distress syndrome?
Definition
Insulin decreases surfactant production
Term
What are the key mutagens found in cigarette smoke that cause lung cancer?
Definition
Polycyclic aromatic hydrocarbons and arsenic
Term
What are the key risk factors for lung cancer?
Definition
Cigarette smoke, radon and asbestos
Term
What is the key finding on imaging for lung cancer? What happens after you find this key finding?
Definition
Coin lesion: solitary nodule. You must look back at previous X-rays and see if it was there before or if it's changed. You must biopsy it next.
Term
What are two benign causes of "coin lesions" seen on X-ray?
Definition
1. Granuloma (often Tb or fungus {Histoplasmosis}), 2. Bronchial hamartoma (bening tumor made up of LUNG tissue and CARTILAGE)
Term
What are the paraneoplastic syndromes associated w/ Small (oat) cell carcinoma?
Definition
ADH: SIADH, ACTH: Cushing's, Lambert-Eaton syndrome
Term
Which neuroendocrine cells does Small (oat) cell carcinoma stem from?
Definition
Kulchitsky cells
Term
What is the paraneoplastic syndrome associated w/ Squamous cell carcinoma?
Definition
May produce parathyroid homrone --> hyperkalemia
Term
What is the characteristic histology of Squamous cell carcinoma?
Definition
Keratin pearls or intercellular bridges
Term
What is the most common tumor in non-smokers as well as in female smokers?
Definition
Adenocarcinoma
Term
What is the location of the following lung tumors: Small cell, squamous cell, adenocarcinoma, large cell, bronchioloalveolar carcinoma
Definition
Small cell: central , squamous cell: central , adenocarcinoma: peripheral, large cell: central or peripheral , bronchioloalveolar carcinoma: peripheral
Term
Which lung cancer classicaly presents as a pneumonia-like consolidation on imaging and has an excellent prognosis?
Definition
Bronchioloalveolar carcinoma
Term
Which type of cell does a Bronchioloalveolar carcinoma arise from?
Definition
Clara cells
Term
Which lung tumor is chromogranin positive?
Definition
Carcinoid tumor: well-differented neuroendocrine tumor
Term
Which type of lung tumor classically forms a polyp-like mass in the bronchus?
Definition
Carcinoid tumor
Term
What is more common to the lung: primary tumor or metastasis?
Definition
Metastasis
Term
Where does lung cancer love to metastasize?
Definition
Adrenals
Term
Which two lung cancers are NOT related to smoking?
Definition
Bronchioloalveolar carcinoma and carcinoid tumor
Term
What is a Pancoast tumor and what can it lead to?
Definition
It is a tumor in the apex of the lung. It can lead to Horner's syndrome
Term
What is Horner's syndrome?
Definition
This is due to compression of the sympathetic chain due to a Pancoast tumor. It is characterized by: ptsosis (drooping eyelid), miosis (pinpoint pupil), and anhidrosis (no sweating)
Term
What type of pneumothorax shifts the trachea to the side of the collapse?
Definition
Spontaneous penumo
Term
What type of pneumothorax shifts the trachea to the opposite side of the collapse?
Definition
Tension pneumo
Term
What does mesothelioma present with? What is it highly associated with?
Definition
It presents w/ recurrent pleural effusions; asbestos
Term
Why does cleft palate occur?
Definition
Failure of facial prominences to fuse.
Term
In what vasculitis is this triad seen? Triad: aphthous ulcers, genital ulcers, and uveitis
Definition
Behcet syndrome
Term
Where does HSV-1 lay dormant that will lead to a "cold sore"?
Definition
Trigenimal nerve ganglia
Term
What are the risk factors for squamous cell carcinoma of the oral mucosa?
Definition
Tobacco and alcohol
Term
Where is the location of Hairy leukiplakia and what virus is it associated with?
Definition
Patch that arises on the lateral tongue; seen in AIDS patients that have EBV/
Term
Which one is most suggestive of squamous cell dysplasia? Erythroplakia or leukoplakia?
Definition
Erythroplakia
Term
What are clinical features of MUMPS infection?
Definition
Bilateral inflamed parotid glands, orchitis, pancreatitis and asceptic meningitis
Term
What is the most common tumor of the salivary glands? What is its composition? What is the rate of recurrence?
Definition
Pleomorphic adenoma; made up of stroma and epithelial tissue. High rate of recurrence (super high yield)
Term
If a patient had a parotid mass for many years then all of a sudden presents w/ signs of facial nerve palsy, what is the most likely explanation for his symptoms?
Definition
His pleomorphid adenoma transformeed into a carcinoma (this is rare, but can happen)
Term
What is the 2nd most common benign tumor of the salivary gland? What is the characteristic histology of this tumor?
Definition
Warthin's tumor: Cystic tumor w/ lymph node tissue. It has abundnatn lymphocytes and germinal centers
Term
What is the most common malignant tumor of the salivary gland?
Definition
Mucoepidermoid carcinoma.
Term
What is the most common variant of tracheoesophageal fistula?
Definition
Proximal esophageal atresia w/ the distal esophagus arising from the trachea
Term
How will an infant w/ the most common variant of tracheoesophageal fistula present?
Definition
Presents w/ vomiting, polyhydramnios (Excess amniotic fluid), abdominal distension (b/c of air coming into the stomach), and aspiration
Term
What are the characteristics of Plummer Vinson syndrome?
Definition
Severe iron deficiency anemia, esophageal web (thin protrusion of esophageal mucosa), and beefy-red tongue due to atrophic gastritis.
Term
Where does a zenker diverticulum present?
Definition
Arises above the upper esophageal sphincter at the junction of the esophagus and pharynx
Term
What is Mallory-Weiss syndrome?
Definition
Longitudinal laceration of mucosa at the GE junction. Caused by severe vomiting (alcoholism and bulimia)
Term
What is Boerhaave syndrome?
Definition
Rupture of the esophagus leading to air in the mediastinum and subcutaneous emphysema (air bubbles beneath the skin and hear crackling noise that sound like rice crispies)
Term
Which syndromes(Mallory-Weiss, Esophageal varices) present w/ painful hematemesis and which presents w/ painless hematemesis?
Definition
Painful hematemesi: mallory-weiss syndrome. Painless hematemesis: esophageal varices
Term
What is the most common cause of death in cirrhossis?
Definition
Rupture of esophageal varices
Term
Why does achalasia occur? What type of dysphagia do they have?
Definition
Due to damaged ganglion cells in the myenteric plexus. Dysphagia to botoh liquieds and solids.
Term
What is the "bird-beak"sign on Barium swallow associated with?
Definition
Achalasia
Term
What type of carcinoma are patients with achalasia at higher risk for?
Definition
Esophageal squamous cell carcinomoa
Term
What esohpageal anomaly is associated w/ HIGH lower esophageal sphincter (LES) pressure on esophageal manometry? THIS IS HIGH YIELD
Definition
Achalasia
Term
Why does GERD occur?
Definition
Reflux of acid from the stomach due to reduced LES tone
Term
What are some risk factors for GERD?
Definition
Hiatal hernia, alcohol, tobacco, obesity, fat-rish diet, caffeine
Term
What presents as an "hour-glass" appearance of the stomach?
Definition
Sliding hiatal hernia
Term
What is Barrett esophagus?
Definition
Metaplasia of the lower esophageal mucosa from stratified nonkeratinizing squamous epithelium to NONciliated columnar epithelium w/ goblet cells
Term
What is the most common esophageal worldwide? In the west?
Definition
Worldwide: squamous cell carcinoma, West: adenocarcinoma
Term
What are the risk factors for squamous cell carcinoma of the esophagus?
Definition
Alcohol, achalasia, cigarettes, plummer-vinson syndrome, zenker's diverticulum, very HOT tea, esophageal injury
Term
What is a risk factor for Adenomcarcinoma of the esophagus?
Definition
Barrett esophagus
Term
Which region of the esophagus does SCC and adenocarcinoma arise in?
Definition
SCC: upper 2/3 of the esophagus, Adenocarcinoma: lower 1/3 of the esophagus
Term
If the carcinoma involves the upper 1/3, or middle 1/3, or lower 1/3 of the esophagus, what lymph nodes will be involved?
Definition
Upper 1/3: cervical nodes. Middle 1/3: mediastinal or tracheobronchial nodes , Lower 1/3: celiac and gastric nodes
Term
What is the difference b/w gastroschisis and omphalocele?
Definition
Omphalocele is covered by peritonoeum and amnion of the umbilical cord, whereas gastroschisis isn't covered by peritoneum.
Term
When does pyloric stenosis commonly present (at birth, after birth)?
Definition
It normally presents about 2 weeks after bith.
Term
How does pyloric stenosis present?
Definition
Projectile NON-BILIOUS vomiting, visible peristalsis, olive-like mass in the abdomen.
Term
What is the difference b/w a Curling ulcer and a Cushing ulcer (examiners love to test on this)? What does these increase the risk for?
Definition
Curling ulcer: hypovolemia leads to decreased blood supply. Cushing ulcer: due to increased intracranial pressure --> increased stimulation of vagus nerve leads to increased acid production (Ach is released and that binds to parietal cells). They both increase the risk for actue gastritis!
Term
What are risk factors for acute gastritis?
Definition
Severe burn (Curling ulcer), NSAIDs (decreased PGE2), heavy alchol consumption, chemotherapy, increased ICP (Cushing's ulcer), Shock
Term
What are the two types of chronic gastritis?
Definition
Chroninc autoimmune gastritis (type A) and chornic H. pylori gastritis (Type B)
Term
What antibodies are associated w/ Chronic autoimmune gastritis?
Definition
Antibodies against parietal cells and or intrinsic factor
Term
What type of hypersensitivity is chronic autoimmune gastritis?
Definition
Type IV since this disease is mediated by T cells.
Term
What is the most common cause of Vitamin B12 deficiency?
Definition
Chronic autoimmune gastritis
Term
What type of carcinoma are patients w/ chronic autoimmune gastritis at risk for ?
Definition
Gastric adenocarcinoma
Term
What part of the stomach does chronic autoimmune gastritis involve?
Definition
The fundus and the body
Term
What is the most common site for chronic H. pylori gastritis?
Definition
The antrum
Term
What is the most common form of gastritis?
Definition
H. pylori induced gastritis
Term
What type of cancers are patietns w/ H. pylori at greatest risk for?
Definition
Gastric adenocarcinoma (intestinal type) and MALT lymphoma (of marginal cell origin)
Term
What is the most common cause of duodenal ulcers? What is a very rare cause that they like to test us on?
Definition
Most common: H. pylori. Rare: ZE syndrome
Term
What type of ulcer am I? Pain is GREATER w/ meals and there is weight loss
Definition
Gastric ulcer
Term
What type of ulcer am I? Pain is DECREAED w/ meals and there is weight gain
Definition
Duodenal ulcer
Term
What is the most common cause of gastric ulcers? What is the 2nd most common cause?
Definition
Most common: H. Pylori (70%), 2nd most common: NSAIDs
Term
What are the two types of gastric adenocarcinoma?
Definition
Intestinal and diffuse type
Term
What are the differences b/w intestinal and diffuse types of gastric adenocarcinomas?
Definition
Intestinal: resembles the colonic adenocarcinoma and it presents as a large, irregular ulcer w/ head up margins usually at the lesser curvature of th antrum (like a gastric ulcer). Diffuse type: characterized by SIGNET cells (cells w/ the nucleus pushed off to the side b/c of increased mucus production by that cell); desmoplasia resulting in thickening of the stomach wall (linitis plastica)
Term
What type of GI cancer is associated w/ blood type A?
Definition
Gastric adenocarinoma
Term
Whar are the risk factors for intestinal gastric adenocarcinoma?
Definition
Intestinal metaplasia (due to H. pylori or autoimmune gastritis), nitrosamines in smoked foods (japan), and blood type A
Term
What carcinoma is associated w/ acanthosis nigricans or Leser-Trelat sign?
Definition
Gastric adenocarcinoma
Term
What lymph node does gastric adenoma carcinoma drain too? What's the fancyname for it?
Definition
Supraclavicular LN = Virchow's nodes
Term
What are the distant mets most commonly involved in gastric adenocarinoma?
Definition
Sister Mary Joseph's nodules: Subcutaneous periumbilical mets; Krunkenberg's umor: bilateral mets to ovaries (abundant Signet cells)
Term
What syndrome is duodenal atresia associated with?
Definition
Down syndrome
Term
What are clinical features of duodenal atresia?
Definition
Polyhydramnios, "double bubble" sign , bilious vomiting
Term
Name a true diverticulum
Definition
Meckel Diverticulum
Term
What are the rules of 2 of Meckel Diverticulum?
Definition
Seen in 2% of the pupulation, 2 inches long and located in the small bowel w/in 2ft of the ileocecal valve, can presnt at first 2yrs of life, 2 mucosa: pancreatic and gastric
Term
What is associated w/ a "coffee bean" sign on x-ray?
Definition
Volvulus
Term
What is the most common location of a volvulus in an eldery patient vs young adult?
Definition
Elderly: sigmoid colon. Young adult: cecum
Term
What is associated w/ "currant jelly stools"?
Definition
Intussusception
Term
What are the classic antibodies found in Celiac disease?
Definition
IgA against endomysium, tTG (tissue transglutaminase), or gliadin. IgG Abs are also present and are useful for dx in patients w/ IgA deficiency
Term
What is dermatitis herpetiformis associated with?
Definition
Celiac disease
Term
What is the duodenal biopsy look like in a patient w/ Celiac disease?
Definition
Flattening of the villi, hyperplasia of crypts and increased intraepithelial lymphocytes. The damage is MOSTLY in the duodenum and the jejum and ilium are less involved.
Term
What should be on your DDx in a patient w/ refractory Celiac disease?
Definition
Smal bowel carcinoma and T cell lymphoma
Term
What parts of the small intestine are preferentially involved in Tropical sprue? What is the vitamin deficiency that they can develop?
Definition
The jejunum and ilieum. Vit B12 def.
Term
What causes Whipple disease?
Definition
Tropheryma whippelii
Term
What is the most common site of Carcinoid tumor in the GI tract?
Definition
Small intestine
Term
What does the carcinoid tumor?
Definition
Serotonin
Term
When do you get carcinoid syndrome from a carcinoid tumor of the GI tract?
Definition
When the tumor has metastasized to the liver and it has seeded in the hepatic vein --> carcinoid hert diease
Term
What is carcinoid syndrome?
Definition
Bronchospasm, diarrhea and flushing of skin
Term
why doesn’t a partient w/ carcinoid syndrome get left sided vavular lesions?
Definition
B/c the lung contains monoamine oxidase (MOA) which metabolizes the serotonin
Term
What is carcinoid heart disease?
Definition
It is characterized by right-sided valvular fibrosis (increased collagen) --> triscuspid regurgitation and pulmonary valve stenosis
Term
What is the wall involvement in UC vs CD?
Definition
UC: mucosal and submucosal ulcers. CD: full-thickness inflammation w. knife-like fissures
Term
Where is the location of UC vs CD?
Definition
UC: begins in the rectum --> goes up to the cecum. CD: Anywhere along the GI tract; get "skip lesions". Most common site is the terminal ileum
Term
What is the key histological finding of UC vs CD?
Definition
UC: Crypt abscesses w/ neutrophils. CD: lymphoid aggregates w/ granulomas
Term
What has a "lead pipe" sign on imaging?
Definition
Ulcerative colitis
Term
What has a "string-sign" on imaging?
Definition
Crohn disease
Term
What are the complications associated w/ UC?
Definition
Toxic megacolon and carcinoma (risk is based on extent of colonic involvement and duration of disease)
Term
What diseases are positive for p-ANCA?
Definition
Microscopic plyangitis, Churg-Strauss and ulcerative colitis
Term
Does smoking protect against UC or CD?
Definition
UC
Term
What are the symptoms of UC vs CD?
Definition
UC: LLW pain (rectum) w/ bloody diarrhea. CD: RLW (ielum) w/ non-bloody diarrhea
Term
Which IBD is associated w/ a cobblestone appearance? What does this represents?
Definition
CD; it represents attempts to heal the intestine
Term
What are the associations of CD?
Definition
Ankylosing spondylitis, sacroilitis, migratory polyarthritis, erythema nodosum, and uveitis
Term
Which IBD has an increased risk of calcium oxalate nephrolithiasis?
Definition
CD
Term
What syndrome is Hirschsprung disease associated with?
Definition
Down syndrome
Term
Why does Hirschsprung disease occur?
Definition
Congenital failure of ganglion cells (neural crest0derived) to descendinto the myenteric and submucosal plexus
Term
what type of biopsy is needed to prove that the patient has Hirschprung's disease?
Definition
Rectal suction biopsy to really look at the ganglion cells
Term
Where is the most common location of a colonic diverticulum? Where does this arise?
Definition
Most common location: sigmoid colon. Arises where the vasarecta traverse the muscularis propria (weal point in the colonic wall)
Term
What are the high yield complications w/ colonic diverticula?
Definition
Rectal bleeding (hematochezia), diverticulitis (left sided appendicitis like symptoms), fistula (colovesicular fisulta-air or stool in the urine)
Term
What is the most common cause of ischemic collitis?
Definition
Atherosclerosis of the SMA
Term
What is the most comon type of colonic polyp? 2nd most common type? Which one is pre-malignant?
Definition
Most common: hyperplastic (no malignant potential), 2nd most common: adenomatous polyp (this is pre-malignant)
Term
What tumor suppressor gene presents a risk for colon cancer? What chromosome is the gene found on?
Definition
APC on Chromosome 5
Term
What mutation allows for the formation of the polyp and which gene leads to progression of the carcinoma?
Definition
K-ras mutation allows for formation of the polyp. P53 mutation and increased expression of COX allows for progression to carcinoma
Term
Is apirin protective of colon cancer?
Definition
Yes b/c it inhibits the production of COX and therefore slows down the progression from adenoma to carcinoma.
Term
Which polyps have a higher risk of progressing to carcinoma?
Definition
Size > 2cm, sessile growth and villous histology ("the villian in colon cancer")
Term
What is Gardner's syndrome?
Definition
FAP with fibromatosus and osteomas (usually arises in the skull)
Term
What is Turcout syndrome?
Definition
FAP w/ CNS tumors (medulloblastoma and glial tumors)
Term
What is Peutz-Jegher's syndrome? What carcinomas are they at higher risk for?
Definition
Hamatomatous (benign) polyps throughout the GI tract and mucocutaneous hyperpigmentation (freckle-like spots) on lips, oral mucosa and genital skin; this is AD disorder. Increased risk for colorectal, breast and gynecological cancers.
Term
What are the 3 types of cancer that patients w/ HNPCC are at risk for?
Definition
Colorectal, ovarian and endometrial carcinoma.
Term
What is HNPCC?
Definition
it is due to an inherited mutation in DNA mismatch repari enzymes.
Term
The right vs the left side of the colon are associated w/ which cancer pathways? Choices are either adenoma carcinoma sequenc or microsatellite instability.
Definition
Right: adenoma carcinoma sequence (usually APC) vs the left: microsatellite instability (usually HNPCC)
Term
What is the tumor marker associated w/ colorectal cancer? It is a good marker for dx or recurrence?
Definition
CEA; recurrence
Term
What type of necrosis will you get in acute pancreatitis?
Definition
Liquifactive necrosis of the pancreas itself and then fat necrosis of peripancreatic fat.
Term
What are good serum markers for acute pancreatitis but not chronic pancreatitis.
Definition
Serum lipase and amylase
Term
What are the major risk factors for pancreatic carcinoma?
Definition
Smoking and chronic pancreatitis
Term
What are the major risk factors for chronic pancreatitis?
Definition
Alcohol and cystic fibrosis.
Term
What is the serum tumor marker for pancretic adenocarcinoma?
Definition
CA 19-9
Term
What are the clinical features of pancreatic carcinoma?
Definition
Epigastric abdominal pain and weight loss; obstructive jaundice w/ pale stools and palpable gallbladder (associated w/ tumros that arise in the head of the pancreas); secondary DM (associated w/ tumors in the body or tail of the pancreas), pancreatitis, and migratory thrombophlebitis (Trousseau sign)
Term
Where is the most common location of a pancreatic carcinoma?
Definition
The head of the pancreas
Term
What are the two types of gallstones? Which one is most common?
Definition
Cholesterol (most common) and bilirubin
Term
What are the risk factors for cholesterol stones?
Definition
Age (40s), estrogen (female gender, obesity, multiple pregnancies and oral contraceptives), clofibrate, Native American ethnicity, Crohn disease, and cirrhosis
Term
Which type of gallstone is radiolucent and which is radiopaque?
Definition
Radiolucent: cholesterol; Radiopaque: bilirubin
Term
What increases the risk of bilirubin stones?
Definition
Extravascular hemolysis and bilary tract infection.
Term
How does acute cholecystitis present on physical exam?
Definition
Presents w/ right upper quadrant pain, often radiating to right scapula.
Term
What gallbladder disease is associated w/ Rokitansky-Aschoff sinus? What is it?
Definition
Chronic cholecystitis; characterized by herniation of gallbladder mucosa into the muscular wall.
Term
What is a porcelain gallbladder a late complication of? Why do you have to remove it?
Definition
Chronic cholecystitis; it increases your risk of carcinoma
Term
What is your dx of an elderly woman who presents w/ cholecystitis?
Definition
Gallbladder carcinoma
Term
Why is the urine dark in extravascular hemolysis or ineffective erythropoiesis?
Definition
Dark urine due to icnrease in urine urobilinogen (and NOT Unconjugated bilirubin since UCB is NOT water soluble
Term
What can lead to increased unconjugated bilirubin?
Definition
extravascular hemolysis or ineffective erythropoiesis, physiologic jaundice of the newborn, Gilberty syndrine, Crigler-Najjar syndrome, viral hepatitis
Term
What can lead to an increase in conjugated bilirubin?
Definition
Dubin-Johnsone syndrome, Rotor syndrome, Biliary tract obstruction )obstructive jaundice), viral hepatitis.
Term
What is the treatment of physiological jaundice of the newborn and what it it's purpose?
Definition
Phototherapy; it makes the unconjugated bilirubin water soluble so that it can leak out into the urine (but it does NOT conjugate it)
Term
What's the difference b/w Gilbert syndrome and Crigler-Najjar syndrome (I)?
Definition
GS: mildly low UGT activity (AR) while CN has absence of UGT.
Term
What cause of jaundice has increase CB and a black liver?
Definition
Dubin-Johnson syndrome
Term
Why does the patient get both increased CB and UCB with viral hepatitis? Why is the patient's urine dark?
Definition
CB: b/c of damage to the small bile ductules, UCB: b/c of damage to the hepatocytes and they can't conjugate the bilirubin. Dark urine b/c of increased CB.
Term
Which liver enzymes are elevated in viral hepatitis?
Definition
ALT > AST.
Term
Which two hepatitis viruses are transmitted fecal-oral?
Definition
HAV and HVE
Term
What happens if a pregnant woman has HEV?
Definition
It is associated w/ fulminant hepatitis (liver failure w/ massive liver necrosis).
Term
What is the first serological marker to rise in the acute phase of Hepatitis B infection? What does it mean if it is still present for more than 6 months?
Definition
HBsAg, if present for >6 months then that defines a chronic state
Term
What does HBeAg indicate?
Definition
Infectivity
Term
What does HDV require for inection?
Definition
It is dependent on HBV for infection
Term
What is more severe: a coinfection or a superinfection of HBV and HDV?
Definition
A superinfection is more severe. It is when the patient first got HVB and then got HDV
Term
What mediates fibrosis in cirrhosis? What does this cell secrete?
Definition
Stellate cells secrete TGF-Beta
Term
What are clinical signs of excess estrogen in a patient w/ cirrhosis?
Definition
Gynecomastia, spider angiomata, and palmar erythema
Term
What does decreased protein synthesis lead to in cirrhosis?
Definition
Hypoalbuminemia w/ edema; coagulopathy due to dec protein synthesis of clotting factors (def of def is followed by PT).
Term
What are Mallory bodies?
Definition
Damaged intermediate filaments (cytokeratin filaments)
Term
What are the elevated liver enzymes in alcohol related liver disease?
Definition
AST > ALT
Term
What liver enzyme is preferentially increased when there is alcohol based damage?
Definition
AST
Term
In hemochromatosis, what mediates tissue damage?
Definition
Generation of free radicals
Term
What is the mutation in primary hemochromatosis?
Definition
Mutation in the HFE gene, usually C282Y (cystein ie replaced by tyrosine at the amino acid 282)
Term
What am I? Increased ferritin, decrease TIBC, increased serum iron, and increased % saturation
Definition
Hemochromatosis
Term
What is the classic triad in hemochromatosis?
Definition
Cirrhosis, secondary diabetes and bronze skin (Bronze diabetes)
Term
What type of carcinoma do patients w/ hemochromatosis have an increased risk of?
Definition
Hepatocellular carcinoma
Term
What am I? Increase urinary coppe, decrease serum ceruloplasmia, and increased copper on liver biopsy
Definition
Wilson disease
Term
What is the treatment for Wilson diease? What defect is it associated with?
Definition
Tx: D-penicillamine (chelates copper). WD is associated w/ autosomal recessive defect (ATP7B gene) in ATP mediated hepatocyte copper transport.
Term
What auto-Ab is present in primary biliary cirrhosis?
Definition
Antimitochondrial antibody
Term
What disorder is associated w/ ulcerative collitis, is p-ANCA, presents w/ obstructive jaundice and increases the patietns' risk for cholangiocarcinoma?
Definition
Primary sclerosing cholangitis
Term
What disorder has a) Inflammation and fibrosis of both the intrahepatic and extrahepatic bile ducts, b) autoimmune granulomatous destruction of intrahepatic bile ducts?
Definition
a) Primary sclerosing cholangitis, b) Primary biliary cirrhosis
Term
What is Reye syndrome?
Definition
Fulminant liver failure and encephalopathy in kids w/ viral illness who tooke aspirin --> it is likely related to MITOCHONDRIAL damage of hepatocytes
Term
What are the risk factors for hepatocellular carcinoma?
Definition
Chronic hepatitis (HBC, HCV), cirrhosis, aflatoxins derived from Aspergillus (incudce p53 mutations)
Term
What is the serum tumor marker for hepatocellular carcinoma?
Definition
alpha-fetoprotein
Term
What biliary tract disease is associated w/ concentric "onion skin" bile duct firbosis --> alternating strictures and dilation w/ "beading" of intra and extrahepatic bile ducts on ERCP?
Definition
Primary sclerosing cholangitis
Term
What is the most common congenital renal anomaly? Why does this occur?
Definition
Horeshoe kidney. It gets caught on the inferior mesenteric artery root.
Term
What syndrome is associated w/ bilateral reanal agenesis, flat flace w/ low set ears and developmental defect of the extremities? Is this compatible w/ life?
Definition
Potter syndrome; incompatilbe w/ life
Term
How do you distinguish b/w dysplastic kidney from inherited PKD?
Definition
Dysplastic kidney is NOT inherited.
Term
What are the two presentations of polycystic kidney disease?
Definition
Autosomal recessive (infantile) and autosomal dominant (adult form)
Term
What should you suspect if there is a Family hx of renal disease, death occuring b/c of renal disease or brain hemorrhage?
Definition
ADPKD; these patients get cyst in the kidney, liver and brain. Due to mutation in APKD1 or APKD2 genes.
Term
If a newborn presents w/ Potter sequence and portal HTN, what should you suspect?
Definition
ARPKD; associated w/ congenital hepatic fibrosis and hepatic cysts
Term
Shrunken kidneys w/ cysts in the medulla of the kidney
Definition
Medullary cystic kidney disease
Term
What leads to prerenal azotemia? What leads to postrenal azotemia?
Definition
Prerenal: decreased blood flow (decreased GFR, azotemia, oliguria). Postrenal: obstruction of uinary tract downstream from the kidney (decreased GFR, azotemia, and oliguria)
Term
What happens in the early stage versus long-standing obstruction of postrenal azotemia?
Definition
Early stage: serum BUN:Cr ratio >15, tubular function remains intact Fena <1% and urine osm >500 mOsm.kg). Long-standing obstruction: serum BUN"Cr ratio <15, decreased reabsorption of Na (FENa >2%) and inability to concentrate urine (urine osm < 500 mOsm/kg)
Term
What is the most common cause of acute renal failure (intrarenal azotemia)?
Definition
Acute tubular necrosis
Term
What is associated w/ brown, granlar casts seen in the urine, Elevated BUN and creatinine, hyperkalemia w/ metabolic acidosis?
Definition
Acute tubular necrosis
Term
What are the common causes of nephrotoxic-acute tubular necrosis?
Definition
Aminoglycosides (most common), heavy metals (lead), myoglobinuria (from crush injury to the muscle), ethylene glycol (associated w/ oxalate crystals in the urine), radiocontrast dye, and urate (tumor lysis syndrome)
Term
What are the causes of acute interstital nephritis?
Definition
NSAIDs, Penicillin and diuretics
Term
What is pathognomonic for acute interstitial nephritis? What may AIN progress to?
Definition
Eosinophils! Renal papillary necrosis.
Term
What are the common causes of renal papillary necrosis?
Definition
Chronic analgesic abuse, DM, sickle cell trait or disease, severe acute pyelonephritis
Term
What disorders can nephrotic syndrome result in?
Definition
Hypoalbuminemia (pitting edema), hypogammaglobulinemia (increased risk of infection), hypercoagulable state (due to loss of antithronbin III), hyperlipidemia and hypercholesterolemia (may result in fatty casts in urine)- examiners like to test on this!!
Term
Which nephrotic syndrome is associated w/ Hodgkin's lymphoma?
Definition
Minimal change disease (MCD)
Term
What is the key finding on EM of minimal change disease? What immune cell mediates the damage to the kidney?
Definition
Effacement of foot processes; Damage is mediated by cytokines from T cells.
Term
What kidney syndrome has a spike and dome" finding with subepithelial depositions on electron microscopy?
Definition
Membranous glomerulonephritis (difuse membranous glomerulopathy)
Term
What kidney syndrome is associated w/ effacement of the foot process on electron microscopy in Hispanics and African Americans?
Definition
Focal segmental glomerulosclerosis (FSGS)
Term
What is the most common golmerular disease in HIV patients?
Definition
Focal segmental glomerulosclerosis (FSGS)
Term
What is the msot comon cause of adult nephrotic syndrome?
Definition
Membranous glomerulonephritis (difuse membranous glomerulopathy)
Term
What is the type of nephrotic syndrome that is associated w/ SLE?
Definition
Membranous glomerulonephritis (difuse membranous glomerulopathy)
Term
Membranous glomerulonephritis (difuse membranous glomerulopathy) H&E and IF description?
Definition
H&E: thick glomerular basement membrane. IF: immune complex deposition (granular)
Term
What nephrotic syndrome appears with a "tran track" apperance with thick glomerular basement membrane on H&E?
Definition
Membranoproliferative glomerulonephritis
Term
Where are the deposits in type I and II Membranoproliferative glomerulonephritis?
Definition
Type I: deposits under the endothelial cells (subendothelial); more often associated w the formation fo tram tracks; associated w/ HBV and HCV. Type II: they have intramembranous dense deposits; these patients hasve C3 nephrtic factor which is an autoantibody that stabilizes C3 convertase.
Term
In what nephrotic syndrome will you see Kimmelstiel-Wilson lesions and what are they?
Definition
Diabetic glomerulonephropathy; they're sclerosis of the mesangium
Term
What is the most commonly involved organ in systemic amyloidosis?
Definition
The kidney; will see apple-green birefringenece under polarized light after staining w/ congro red.
Term
What are the characteristics of Nephritic syndrome?
Definition
Inflammatory process; hematuria and RBC casts in the urine, associated w/ azotemia, oliguria, hypertension (due to salt retention), and proteinuria (<3.5g/day)
Term
Name the nephritic syndrome: EM: subepithelial immune complex (IC) humps, LM: glomeruli enlarged and hypercellular, neutrophils, "lumpy-bumpy" apperance, IF: granular appearance due to IgG, IgM and C3 deposition along GBM and mesangium
Definition
Acute Poststreptococcal pneumonia
Term
What defines the nephritogenic strains of Streptococcus?
Definition
The M protein
Term
What is the composition of the crescents in Rapidly progressive glomerulonephritis (RPGN)?
Definition
Crescents: fibrin and macrophages
Term
What 3 diseases can results in Rapidly progressive glomerulonephritis (RPGN)?
Definition
Goodpasture syndrome, Wegener's granulomatosis and microscopic polyangitis
Term
What is the IC pattern for Goodpasture syndrome that led to Rapidly progressive glomerulonephritis (RPGN)?
Definition
Linear (anti-basement membrane antibody)
Term
What is the IC pattern for Wegener's granulomatosis and microscopic polyangitis that led to Rapidly progressive glomerulonephritis (RPGN)?
Definition
Negative IF (Pauci-immune)
Term
p-ANCA is associated with? c-ANCA is associated with?
Definition
c-ANCA: Wegener's syndrome, p-ANCA: Microscopic polyangitis, Churg strauss
Term
How do you distinguish Churg Strauss from Microscopic polyangitis?
Definition
CS has: granulomatous inflammtion, eosinophilia and asthma
Term
What is Alport syndrome? What are the effects of it on the glomerular basement membrane?
Definition
Inherited defect in type IV collagen, most commonly X-linked. Get thinning and splitting of the GBM --> hematuria. Disease also associated w/ sensory hearling loss and ocular disturbances.
Term
What is IgA nephropathy (Berger disease)?
Definition
It is related to Henloch-Schonlein Purpura. Get IgA mesangial IC deposits.
Term
What is the most common cause of death in SLE?
Definition
Diffuse proliferative glomerulonephritis
Term
What is the seen on H&E, EM for diffuse proliferative glomerulonephritis?
Definition
H&E: "wire looping" of capillaries. EML subendothelial and sometiems intramembranous IgG based Ics often w/ C3 deposition
Term
What are the most common pathogens involved in pyelonephritis?
Definition
E. coli (90%), Klebsiella species, Enterococcus faecalis
Term
When would you see a white blood cell casts?
Definition
Pyelonephritis
Term
When does "thyroidization " of the kidney occur?
Definition
In chronic pyelonephritis; Atropic tubules containing eosinophilic proteinaceous materal that resembles thryoid follicles
Term
What is the most common type of nephrolithiasis? What is the treatment?
Definition
Calcium oxalate and or calcium phosphate. Tx: hydrochlorothiazide (Calcium sparring diuretic)
Term
What is the 2nd most common type of nephrolithiasis?
Definition
Ammonium magnesium phosphate
Term
What is a radiolucent kidney stone? What is the most common type of stone seen in patients w/ gout? What is the treatment?
Definition
Uric acid; Tx: hydration and alkalinization of urine
Term
What is the most common cause of kidney stones seen in children? Tx?
Definition
Cysteine stones. Tx: hydration and alkalinization of urine.
Term
Which part of the kidney makes erythropoietin?
Definition
The renal peritubular interstitial cells
Term
What is the composition of a staghorn calculi in an adult vs a child?
Definition
Adult: Ammonium magnesium phosphate, Child: cysteine
Term
What type of cancer are patients on dialysis most likely to get in the shrunken end-stage kiendy?
Definition
Renal cell carcinoma
Term
Where does renal cell carcinoma arise from? What is the classic triad? What syndrome is it associated it?
Definition
It arises from the kidney tubule. Triad: hematuria, palpable mass and flank pain. It is associated w/ von-Hippel-Lindau syndrome and gene deletion on chromosome 3.
Term
Name some paraneoplastic syndromes that Renal Cell Carcinoma is associated with?
Definition
EPO (leading to reactive polycythemia vera), renin (leading to HTN), PRHrP (leading to hypercalcemia), ACTH (leading to Cushing's), or prolactin (decreased libido, galactorrhea)
Term
Can renal cell carcinoma present w/ right or left sided varicocele?
Definition
Left sided varicocele since the RCC can metastasize to the left renal vein.
Term
What is the most common variant of Renal cell carcinoma?
Definition
Clear cell type
Term
what is the gene deletion associated w/ Renal Cell Carcinoma and what does it lead to?
Definition
Loss of VHL (Chromosome 3) tumor suppressor gene --> leads to increased IGF-1 (promotes growth) and increased HIF transcription factor (increases VEGF and PDGF --> promotes angiogenesis).
Term
Patients w/ von-Hippel Lindau syndrome are at increased risk for what cancers and why?
Definition
Hemangioblastoma of the cerebellum and renal cell carcinoma b/c of inactivation of VHL gene. Pheochromocytoma
Term
What is the most malignatn renal tumor in children?
Definition
Wilm's tumor
Term
What is the gene mutation associated w/ Wilm's tumor? What is WAGR syndrome? What is Beck-with Wiedemann sndrome?
Definition
WT1 mutation. WAGR syndrome: wilm's tumor, aniridia, genital abnormalities and mental and motro retardatin. Beckwith-wiedemann syndrome: wilms tumor, neonatal hypoglycemia, muscular hemihypertrophy and organomegaly (including tongue)
Term
What is the cell composition of a Wilms tumor?
Definition
Blastema (immature kidney mesencyme), primitive glomeruli and tubules, and stroma cells
Term
What is the most common type of lower urinary tract cancer?
Definition
Urothelial (transitional cell) carcinoma
Term
What is the major risk factor for Urothelial (transitional cell) carcinoma?
Definition
Cigarette smoke
Term
What are high risk factors for squamous cell carcinoma of the bladder?
Definition
Chronic cystitis (older woman), Schistosoma hematobium infection (Egyptian male), and long standing nephrolithiasis.
Term
An adenocarcinoma that arises from the urachal remant is present where on the ballder?
Definition
It is present at the DOME of the bladder.
Term
SUPER high yield: what HPV types are associated w/ a condyloma acuminatum? What is the characteristic change of the cell?
Definition
6 and 11; koilocytes
Term
What are the low and high risk HPV strains?
Definition
Low risk: 6, 11. High risk: 16, 18, 31 and 33
Term
Parchment like vulvar skin is a characteristic of? What are other characteristics of this? Is there an increased risk of squamous cell carcinoma?
Definition
Lichen sclerosis; thinning of the epidermis and fibrosis (sclerosis) of the dermis, presents as leukoplakia (white patch). Slightly increased risk of SCC.
Term
What presents as leukoplakia w/ thick, leathery vulvar skin?
Definition
Lichen simplex chronicus
Term
What are the two etiologies of vulvar carcinoma?
Definition
1. HPV-related (HPV 16,18) seen in younger women, 2. Non-HPV related: due to long standing lichen sclerosis that is seen in older women.
Term
What is PAS+, keratin+, and S100-?
Definition
Paget cells
Term
What is PAS=, Keratin-, and S100+?
Definition
Melanoma
Term
What is the embryological origin of the upper 2/3 and the lower 1/3 of the vagina? What type of cells are they lined with?
Definition
Upper 2/3: Mullerian ducts covered w/ columnar epithelium. Lower 1/3: urogenital sinus that is covered w/ squamous epithelium
Term
What is adenosis? Who has an increased incidence of adenosis?
Definition
Focal persistance of columan epithelium in the upper 2/3 of the vagina. It should have been replaced w/ squamous cell epithelium. Females who were exposed to DES in utero have an icnreased incidence of adenosis.
Term
What is a complication of DES-associated vaginal adenosis?
Definition
Clear cell adenocarcinoma: malignant proliferation of glands w/ clear cytoplasm
Term
What is a grape-like mass that a) exhibits cytoplasmic cross-striations and b) is positve for demin and myogenin?
Definition
Embryonal rhabdomyosarcoma
Term
A vaginal carcinoma that occurs at the upper 2/3 of the vagina vs one that occurs at the lower 1/3 of the vagina will go to what regional lymph nodes?
Definition
Upper 2/3: iiliac nodes (external and internal). Lower 1/3: superficial inguinal lymph nodes
Term
What are the exocervix and endocervix lined by?
Definition
Exocervix: nonkeratinizing squamoud epithelium. Endocervix: single layer of columnar cells.
Term
What is the transformation zone of the cervix?
Definition
The junction b/w the exocervix and endocervix.
Term
What is the key place in the lower genital tract (female) that HPV likes to infect?
Definition
The transformation zone of the cervix.
Term
HIGH YIELD: What proteins do high-risk HPV produce? How do they work?
Definition
They produce E6 and E7 proteins that knock out p53 and Rb, respectively. If you knock out p53 then the cell can progress from G1 --> S phase. If you knock out Rb then E2F can go into the nucleus and work as a transcription factor.
Term
What is the molecule that stabilizes the mitochondrial membrane that inhibits the leaking of cytochrome c?
Definition
Bcl2
Term
What are the classical presentations for cervical carcinoma?
Definition
Middle-aged women; presents w/ vaginal bleeding, especially postcoital bleeding or cervical discharge
Term
What is the KEY risk factor cervical carcinoma? What are secondary risk factors?
Definition
KEY risk factor: high risk HPV infection. Secondary: smoking and immunodeficiency (cervical carcinoma is an AIDS defining illness)
Term
What subtypes of cervical carcinoma are HPV driven?
Definition
Squamous cell carcinoma and adenocarcinoma
Term
What is the cellular characteristic of high grade cervical dysplasia?
Definition
Hyperchromatic (dark) nuclei and high nuclear to cytoplasmic ratio.
Term
What is Asherman syndrome? What is the most common cause of it?
Definition
Secondary amenorrhea due to loss of stratum basalis (regenerative layer of the endometrium and it contains the stem cells of the endometrium) and scarring. Most common cause: over-aggressive D&C.
Term
Where are the stem cells of the endometrium?
Definition
They're located in the stratum basalis.
Term
What is a common cause of acute endometritis?
Definition
Usually due to retained products of conception.
Term
What is a characteristic of CHRONIC endometritis? What are some causes?
Definition
Lymphocytes and plasma cells. Causes: retained products of conception, chronic PID, IUD and TB
Term
What is a weird side effect of Tamoxifen on the endometrium?
Definition
It can result in an endometrial polyp since it has pro-estrogenic effects on the endometrim.
Term
What is the leading theory of endometriosis? What are some other theories?
Definition
Leading theory: etrograde menstruation. Others: metaplasia and lymphatic dissemination
Term
In what syndrome would you see a "chocolate" cysts?
Definition
Endometriosis
Term
is the uterus normal or enlarged in endometriosis?
Definition
It is normal in endometriosis. It is ENLARGED in adenomyosis.
Term
What is adenomyosis? What is the common presentation?
Definition
Endometriosis in the myometrium. Presentation: menorrhagia, dysmenorrhea, pelvic pain and uterus that is ENLARGED.
Term
What is the cause of endometrial hyperplasia? How does it present?
Definition
Unopposed estrogen, e.g. obesity, polycystic ovary syndrome, estrogen replacement. Presentation: postmenopausal uterine bleeding.
Term
What is the most common gynecologic malignancy?
Definition
Endometrial carcinoma
Term
What are the two distinct pathways for endometrial carcinoma?
Definition
Hyperplasia and sporadic pathway
Term
What is characteristic of the hyperplasia pathway of endometrial carcinoma?
Definition
The carcinoma arises from endometrial hyperplasia. Histolgy is endometriod (looks like the normal endometrium). Avg age of presentation is 60years.
Term
What is characteristic of the sporadic pathway of endometrial carcinoma? What is the common gene mutation associated w/ this pathway?
Definition
Histology is SEROUS, characterized by papillary structures w/ PSAMMOMA BODY FORMATION. p53 is commonly mutated.
Term
What tumors will you find psammoma bodies?
Definition
Papillary thyroid carcinoma, MESOTHELIOMA, meningioblastoma, sporadic endometrial carcionma (serious kind)
Term
What is a multiple, well-defined, white, whorled mass that may distor and impinge on pelvic structures?
Definition
Leiomyoma (fibroids)- most likely to be benign since there are multiples of them and wince theyr'e whorled.
Term
Leiomyoma vs leiomyosarcoma?
Definition
Leiomyoma: usually pre-menopausal women. They DO NOT becoem leiomyosarcoma. These usually go away after menopause b/c the lack of estrogen makes them shrink. Leiomyosarcoma: usually occurs after menopausal, usually a single lesion w/ necrosis and hemorrage, increased mitotic activity.
Term
What is the most common clinical finding of leiomyoma?
Definition
NOTHING! Clincally asymptomatic for the most part!
Term
What is the role of LH on theca cells?
Definition
It makes the theca cell produce androgen. The androgen then goes to the granulosa cell.
Term
What is the role of FSH on granulosa cells?
Definition
The granulosa cell converts androgen to estradiol. The estradiol cell then hits the oocyte and allows it to mature. The estrogen goes into the endometrium and causes it to proliferate.
Term
What leads to ovulation?
Definition
Estradiol surge induces LH surge which leads to ovulation (marks the beginning of the secretory phase of the endometrial cycle)/
Term
What is the characteristic of Polycystic ovarian disease (PCOD) in terms of hormones?
Definition
Increased LH and low FSH (LH: FSH >2)
Term
What does increased LH do to a woman in PCOD?
Definition
Increased LH --> excess androgen production from the theca cells --> hirsutism. Androgen is concerted to estrone in adipose tissues.
Term
What are the two most common subtypes of surface epithelial tumors?
Definition
Serous tumors: full of watery fluid. Mucinous tumors: full of mucus-like fluid
Term
What type of ovarian and fallopian tubes are BRCA1 mutation carriers at increased risk for?
Definition
Serous carcinoma o fthe ovary and fallopian tubes
Term
What is a Brenner tumor?
Definition
Composed of bladder-like(urothelium) epithelium and are usually benign.
Term
What are the most common type of ovarian tumors? What is the 2nd most common type of ovarian tumor?
Definition
Most common: Surface epithelia tumors, 2nd: germ cell tumors
Term
What is a useful serum tumor marker for Ovarian cancer?
Definition
CA-125
Term
What is the most common type of germ cell tumor in females?
Definition
Cystic teratoma
Term
What conferms malignancy in a cystic teratoma in a female?
Definition
Presence of immature tissue (usually neural ectoderm) or somatic malignancy (usually Squamous cell carcinoma of the skin)
Term
What would you place on your differential if a woman in her 20s comes in w/ a mass in her ovaries and signs of hyperthryoidism?
Definition
Struma ovarii, hyperthyroidism, Graves disease
Term
What is the most common malignant germ cell tumor in a female?
Definition
Dysgerminoma
Term
What type of germ cell tumor presents as a mass w/ clear cytoplasm and central nuclei?
Definition
Dysgerminoma
Term
What is the male counterpart of a female dysgerminoma?
Definition
Seminoma
Term
Why does a dysgerminoma have a good prognosis?
Definition
B/c it responds well to radiotherapy/
Term
What is a glomerulus-like strucutre in the context of a germ cell tumor?
Definition
It's called a Schiller-Duval body seen in endodermal sinus tumor.
Term
What is the most common germ cell tumor in children?
Definition
Endodermal sinus tumor
Term
What germ cell tumor is made up of trophoblasts and syncytiotrophoblast? What can be used to detect this tumor? Does it respond well to chemotherapy?
Definition
Choriocarcinoma; high beta-hCG. It does NOT respond well to chemothreapy.
Term
What hormone do Granulosa-theca cell tumors secrete? With are the signs of the secretion of this tumor?
Definition
Estrogen; Estrogen excess: prior to puberty will get precocious puberty; at reproductive age: menorrhagis or metrorrhagia; if postmenopausal: abnornal uterine bleeding.
Term
What type of Sex-cord stromal tumor will containe a REINKE crystal? What hormone will this tumor produce?
Definition
Sertoli-Leydig cell tumor; secret androgen
Term
What is Meigs syndrome? What is the treatment?
Definition
Fibroma (bening tumor of fibroblast) associated w/ plerual effusions ans ascites. Tx: removal of tumor.
Term
What is a krukenberg tumor?
Definition
Metastatic mucinous tumor that involes both ovaries; most commonly due to metastatic gastric carcinoma (diffuse type that will have signet ring cells). If it's bilateral involvement of the ovaries then it's usually b/c of metastases.
Term
What is pseudomyxoma peritonei?
Definition
massive amounts of mucus in the peritoneum due to mucinous tumor of the appendix. Also known as "jelly belly" b/c of all the mucus w/in the abdomen.
Term
What is the most common site of an ectopic pregnancy? What is the key risk factor?
Definition
The lumen of the fallopian tube. Key risk factor: scarring of the tubes (either due to PID or endometriosis).
Term
What is the most common reason for a spontaneous abortion?
Definition
Chromosomal anomalies.
Term
What occurs to the fetus if it is exposed to a teratogen during the first 2 weeks of gestation? Weeks 3-8? Months 3-9?
Definition
First 2 weeks: spontaneous abortion. Weeks 3-8: risk of organ malformation. Months 3-9: risk or organ hypoplasia.
Term
What is placenta previa?
Definition
Implantation of the placena in the lwoer uterine segment
Term
What is placenta abruptia?
Definition
Separation of placent from the decidua prior to delivery of the fetus; common cause of stillbrith
Term
What is placenta accreta?
Definition
Improper implantation of placent into the myometrium w/ little or not intervening decidua. Presents w/ difficult delivery of the placenta and pospartum bleedings. Often results in hyterectomy.
Term
What is the one difference b/w preeclampsia and eclampsia?
Definition
Seizures
Term
In preeclampsia, what type of necrosis will you see in the vessels of the placenta?
Definition
Fibrinoid necrosis
Term
What is HELLP syndrome?
Definition
Hemolysis, Elevated Liver enzymes, and Low Platelets
Term
What is the difference b/w a partial and a complete hydatidiform mole?
Definition
Partial: 69 chromosomes (2 sperm fertilize a normal egg), fetal tissue is present, some villi are hydropic and some are normal, there is minimal risk for choriocarcinoma. Complete mole: 46 chromosomes (Empty ovum fertilized by two sperm or empty ovum fertilized by one sperm that immediately duplicates it's chromosomes), fetal tissue is absent, most villi are hydropic, there is a 2-3% risk for choriocarcinoma.
Term
What clasically presents as a passage of grape-like masses throug the vaginal canal in the 2nd semester (if there is no prenatal care)?
Definition
Hydatiform mole
Term
What is a "snowstorm" apperance on ultrasound?
Definition
Hydatiform mole
Term
What is the treatment for Hydatiform mole?
Definition
Dilatation and curettage, subsequent beta-hCG monitoriing b/c that will screen for the development of choriocarcinoma which usually occurs w/in one year.
Term
What is the difference in response to chemoherpy of choriocarcinomas that arise from a gestational pathway versus one that arises from the germ cell pathway?
Definition
Choriocarcinomas that arises from the gestational pathway RESPONDS WELL to chemo!
Term
What is the difference b/w hypospadias and epispadias?
Definition
Hypo: opening of urethra on inferior (ventral) surface of the penis; due to failure of the urethral folds to close. Epi: opening of urethra on superiro (dorsal) surface of penis; due to abnormal position of the genital tubercle. This has an associtation w/ BLADDER EXSTROPHY.
Term
What serotypes of Chlamydia trachomatis cuase lymphogranuloma venereum?
Definition
Serotypes L1-L3
Term
What are some precursor lesions for squamous cell carcinoma of the penis?
Definition
Bowen disease (in situ carcinoma of the penile shfat or scrotum; presents as leukoplakia), Erythroplasia of Queyrat (in siut carcinoma of the glands) and Bowenoid papulosis (this usually does NOT progress to invasive carcinoma)
Term
What pathogens cause orchitis?
Definition
Young adults: Chlamydia trachomatois (serotype D-K) or Neisseria gonorrhea . Older adults: E. coli and Pesudomonas. Teenage males: Mumps
Term
What vein can Renal cell carcinoma invade leading to a "bag of worms" appearance of the scrotum? What is the "bag of warms"?
Definition
It invades the LEFT renal vein; varicocele
Term
In a hydrocele, in which layer of the scrotum does fluid collect?
Definition
Tunica vaginalis
Term
Describe a seminoma? Does it respond to radiotherapy?
Definition
Large cells w/ cler cytoplasm and central nuclei; homogenous mass with NO hemorrhage or necrosis. Good prognosis; responds to radiotherapy.
Term
If there are increased levels of AFP or beta-hCG and a testicular mass, what would you suspect? What can chemotherapy do to this type of mass?
Definition
Embryonal carcinoma; it may result in differentiation into another type of germ cell tumor. It is a malignatn tumor made of up immature, primitive cells that may produce glands.
Term
What is the most common testicular tumor in children? Hint: it has Schiller-Duval bodies.
Definition
Yolk sac (endodermal sinus) tumor; AFP is characteristcally elevated.
Term
If a male has a choriocarcinoma and he presents w/ both gynecomastia and hyperthyroidism, why is he presenting with these issues?
Definition
Choriocarcinoma secretes beta-hCG and the alpha subunit of hCG is similar to that of FSH, LH and TSH. The hCG can activate TSH receptor --> hyperthyroidism and can activate FSH/LH receptors --> gynecomastia
Term
Are teratomas in males malignant? What can they secrete?
Definition
They're malignant. They can seccrete AFP or beta-hCG
Term
If a man presents w/ a testicular mass that has REINKE crystals on histology what is it? What can this tumor produce and what can in cause if it presents in children vs in adults?
Definition
Leydic cell tumor. It secretes Androgens. Child: precocious puberty. Adults: gynecomastia
Term
What is the most likely cause of a 75 year old man's bilateral testicular mas?
Definition
Lymphoma; usually diffuse Large B cell type.
Term
What are the most common pathogens in acute prostatitis in young adults vs older adults? How does the prostate present?
Definition
Young adult: Chlamydia trachomatis and N. gonorrhoeae. Older adults: E. coli and Pseudomonas. Prosate: tender and boggy
Term
What zone of the prostate does BPH occur? What symptoms can the male present with?
Definition
Central periurethral zone of the prostate. Clinical feaures: problems starting/stopping uring stream; impaired bladder emptying w/ increased risk of infection/hydronephorisis; dribbling; hypertrophy of bladder wall smooth muscle; microscopic hematuria; PSA is slightly elevated
Term
What is the hormoen that drives BPH?
Definition
DHT
Term
Where is the most common location of prostate adenocarcinoma?
Definition
Peripheral posterior zone of the prostate
Term
What is the characteristic look of the cell in prostate adenocarcinoma?
Definition
Glands that have infiltrated the prosate which contains nuclei that have dark nucleoli.
Term
What are two drugs that can be used to reduce testosterone conversion to DHT?
Definition
Continous GnRH (Leuprolide) to shut down the hypothalamus. Flutamide: competitive inhibitor at the androgen receptor.
Term
What is the presentation of a prolactinoma in A) female and B) male?
Definition
A) Female: galactorrhea and amenorrhea. B) Decreased libido and headache. This ithe most common type of pituitary adenoma
Term
What is the treatment for prolactinoma?
Definition
Tx: Bromocriptine or cabergoline; surgery
Term
What is the treatment for a Growth Hormoen cell adenoma? How does it work?
Definition
Octreotide: somatostatin analog that supresses GH release
Term
What is Sheehan syndrome? How can it present?
Definition
Pregnancy related infarction of the pituitary gland. Patients present w/ poor lactation and loss of pubic hair (which is dependent on androgens which arise from increased in LH from the anterior pituitary gland)
Term
What is empty sell syndrome?
Definition
Congenital defect of the sella
Term
Where are ADH and oxytocin made? Where are they released?
Definition
Made in the hypothalamus; released by the posterior pituitary gland
Term
What are the clinical features of Central diabetes insipidus? What is the cause of it ? What is the treatment?
Definition
Due to an ADH deficiency. Clinical: polyuria, polydipsia; HYPERnatremia and HIGH serum osmolality; low urine osmolality and specific gravity. Tx: Desmopressin (ADH analog)
Term
Name two drugs that can cause Nephrogenic diabetes insipidus
Definition
Lithium and demeclocycline
Term
What are the causes of SIADH?
Definition
Causes: Most often due to ectopic production of ADH (small cell carcinoma of the lung); other causes: CNS trauma, pulmonary infections, drugs (cyclophosphamide)
Term
What are the clinical features of SIADH?
Definition
HYPOnatremia and LOW serum osmolality; Mental status changes and seizures: HYPOnatremia --> neuronal sweling and cerebral edema
Term
What is the treatment for SIADH?
Definition
Free water restriction or demeclocycline which blocks the effect of ADH.
Term
What thyroid pathology presents as a) an anterior neck mass and b) which presents at the base of the tongue?
Definition
A) Thyroglossal duct cysts B) Lingual thyroid
Term
What is the most common cause of HYPERthyroidism? Explain it.
Definition
Graves disease. AutoAb (IgG) that stimulates TSH receptor (Type II non-cytotoxic hypersensitivity) which leads to increased production and release of the thyroid hormone
Term
How does HYPERthyroidism lead to an increased BMR and an increase in the activity of the sympathetic NS?
Definition
BMR: due to increased synthesis of Na+K+ATPase. Increased SNS activity: due to increased expression of B1 adrenergic receptors.
Term
What are some key clinlca features of HYPERthyroidism?
Definition
Hypocholesterolemia; HYPERglycemia (due to gluconeogenesis and glycogenolysis)
Term
Why does a patient w/ Graves disease have exophthalmos and pretibial myxedema?
Definition
It is NOT due to the thyroid hormone, but it is due to the fact that these patients have an autoAb against the TSH receptor. TSH activation results in glycosaminoglycan (chondroitan sulfate and hyaluronic acid) buildup, inflammation, fibrosis and edema leading to exophthalmos and pretibial myxedema.
Term
What thyroid disease has "scalloping of the colloid" on histology?
Definition
Graves disease
Term
What are the laboratory findings in Graves disease?
Definition
INcrease total and free T4; Decrease TSH (free T4 downregulates RH receptors in the ant pituitary to decrease TSH release); hypocholesterolemia; increased serum glucose
Term
What is the treatment of thyroid storm?
Definition
Tx: PTU (propylthiouracil), BB, and steroids. PTU: inhibits peroxidase-mediated oxidation, organification and coupling steps of thyroid hormoen synthesis, as well as peripheral conversion of T4 to T3.
Term
What is the most common homrone involved in dyshormonogenetic goiter?
Definition
Congenital defect in the production of thyroid peroxidase
Term
What are some causes of cretinism?
Definition
Maternal hypothyroidism during eraly pregnancy, thyroid agenesis, dyshornomogenetic goiter, and iodine deficiency.
Term
What clinical features does myxedema result in?
Definition
Deepening of the voice and large tongue
Term
What is the most common cause of HYPOthyroidism (where iodine levels are sufficient)?
Definition
Hashimoto's thyroiditis; autoimmune destrone of the thyroid gland. Associated w/ HLA-DR5.
Term
What are the laboratory findings in Hashimoto's thyroiditis?
Definition
Increased TSH, decrease T4, anti-thyroglubulin and anti-microsomal antibodies that are markers of thyroid damage
Term
What are histologic findings in Hashimoto's thyroiditis?
Definition
Hurther cell change (eosinophilic metaplasia of cels that line follicles) and chronic inflammation w/ germinal centers (at increased firsk for B cell (marginal) lymphoma).
Term
A young woman comes in w/ a tender thyroid and she has had previous hx of a recent viral infection. What do you think it could be?
Definition
Subacute granulomatous (De quervain) thyroiditis
Term
A young female patient presents w/ a "hard as wood" thyroid and it has involvement of local structures. What do you think it is?
Definition
Reidel fibrosing thyroiditis
Term
A older patient presents w/ a "hard as wood" thyroid and it has involvement of local structures. What do you think it is?
Definition
Anaplastic carcinoma
Term
What is the mechanism of biopsing the thyroid?
Definition
FNA
Term
What type of thyroid neoplasm involes a benign proliferation of follicles surrounded by a FIBROUS capsule?
Definition
Follicular adenoma
Term
What is the most common type of thyroid carcinoma? What is the major risk factor for it?
Definition
Papillary carcinoma; major risk factor: exposure to ionizing radiation in childhood
Term
What thyroid carcinoma has "Orpha annie eye" nuclei and nuclear grooves and papillae that have psammoma bodies?
Definition
Papillary carcinoma
Term
What is the only wayto distinguish b/w a follicular adenoma and a follicular carcinoma?
Definition
FNA cannot distinguish them! Must see the entire gross specimen. Follicular carcinoma will invade through the fibrous capsule.
Term
What are the 4 carcinomas that like to spread hematogenously and NOT by lymphatics?
Definition
Renal cell carcinoma, Hepatocellular carcinoma, Follicular thyroid carcinoma, and choriocarcinoma
Term
What is the origin of parafollicular C cells? What do they secrete? What cancer are they seen in?
Definition
C cells are neuroendocrine cells that secrete calcitonin. Medullary carcinoma is a malignant proliferation of parafollicular C cells.
Term
What familial syndrome is Medullary carcinoma seen in?
Definition
MEN 2A and MEN 2B
Term
What is the gene mutation that is associatd w/ medullary carcinoma seen in MEN 2A and 2B?
Definition
Mutation in RET oncogene
Term
What aare the actions of PTH?
Definition
Increases bone osteoclast activity --> releasing Ca and phosphate. Increases small bowel absorption of Ca and phosphate (indirectly by activating Vit D). Increases renal calcium reabsorption at the distal tubule and decreaess phosphate reabsorption.
Term
What is the most common cause of primary hyperparathyroidism?
Definition
Parathyroid adenoma
Term
What are the laboratory findings seen in primary hyperparathyroidism?
Definition
Increase serum PTH, increase serum Calcium, Decrease serum phosphate, Increase urinary cAMP (since PTH acts through Gs --> adenylate cylase --> increase cAMP), and increase serum alkaline phosphatase (b/c PTH stimulates osteoblast (that contain the PTH receptors) to lay down bone)
Term
What is the most common cause of secondary hyperparathyroidism?
Definition
Chronic renal failure
Term
What are the lab findings in seconary hyperparathyroidism?
Definition
Increase PTH, decrease serum calcium, increase serum phosphate and incrase alkaline phosphatase
Term
What is Chvostek sign? What is Trousseau sign? What are they associated with?
Definition
Assocaited w/ hypoparathyroidism. Chvosteck sign: tapping on the facial nerve and then eliciting a muscle spasm. Trousseau sign: filling the blood pressure cuff and then eliciting a muscle spasm
Term
What is pseudohypoparathyroidism?
Definition
It is due to an end organ resistance to PTH which is usually due to a defect in the Gs proteinso that the PTH signaling Is impaired. There is an autosomal dominant form that is associated w/ short starue and short 4th and 5th fingers. Labs: hypocalcemia w/ increase PTH levels
Term
Whattype of hypersensitivity is Type I DM? What is the characteristic finding?
Definition
Type IV: autoimmune destruction of beta cells by T lymphocytes. Characterized by inflammation of the islets.
Term
What is the hallmark of Diabetic Ketoacidosis?
Definition
Ketone bodies: beta-hydroxybutyric acid and acetoacetic acid
Term
What is the mechansim of insulin resistance in Type II DM?
Definition
Decreased numbers of insulin receptors
Term
What is seen on histology at the later stages of Type II DM?
Definition
Amyloid deposition in the islets of langerhans.
Term
What is the leading cause of blindess in the world? How does this occur?
Definition
Diabetes. Through osmotic damage since glucoes gets into the lens and gets converted to sorbital via aldose reductase and then it gets trapped through --> cataracts.
Term
What are the 3 P's of MEN1?
Definition
Parathyroid hyperplasia, pituitary adenoma and pancreatic endocrine tumor.
Term
What type of pancreatic endocrine neoplasm is described by Decreased serum glucose levels, increased insulin, increased C-peptide
Definition
Insulinoma
Term
What type of pancreatic endocrine neoplasm is described by achlorhydria and cholelithiasis w/ steatorrhea?
Definition
Somatostatinomas; Achlorhydria: due to inhibition of gastrin. Cholelithiasis: due to inhibition of CCK
Term
What type of pancreatic endocrine neoplasm is described by watery diarrhea, hypokalemia and acholorhydria?
Definition
VIPomas
Term
How does excess cortisol lead to hypertension?
Definition
Cortisol upregulates alpha 1 on the arterioles --> increase effect of NE --> HTN
Term
What are the 3 mechanism by which cortisol is immunosuppresive?
Definition
1. Inhibits phospholipase A2 so you can't generate arachidonic acid (metabolites whicha re essential for inflammation). 2. Inhibits IL-2 which is needed for T cell growth. 3. Inhibits the rlease of histamien from mast cells which is essential for vasodilation and increased permeability.
Term
What is the test used to determine if ACTH production is due to the pituitary adenoma or ectopic production?
Definition
High dose dexamethasone test
Term
What is Cushing's disease?
Definition
Due to ACTH secretion from pituitary adenoma. There is increased ACTH -> increased cortisol production from the adrenal cortex. Will get bilateral adrenal hyperplasia
Term
What is Cushing's syndrome?
Definition
Due to either exogenous or ectopic production of ACTH (i.e. Small cell carcinoma) or to a primary adrenal adenoma, hyperplasia or carcinoa (leads to atrophy of the uninvolved adrenal gland)
Term
What disorder (primary or secondary hyperaldoseronism) leads to high aldosterone and low renin?
Definition
Primary: most commonly due to an adrenal adenoma
Term
What disorder (primary or secondary hyperaldoseronism) leads to high aldosterone and high renin?
Definition
Secondary: seen w/ activation of the renin-angiotensin system
Term
What is the most common cause of congenital adrenal hyperplasia? What are the clinical features associated w/ it?
Definition
Inherited 21-hydroxyalse deficiency; it is required for the production of aldoesterone and corticosteroids. Salt wasting w/ hyponatremia, yperkalemia, and ypovolemia due to lack of aldosterone. Life-threatening hypotension due to lack of cortisol. Clitoral enlargement (females) or precocious puberty (males) due to excess androgens.
Term
What are the clinical features of Congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency?
Definition
Increase in 11-deoxycorticosterone, decrease in aldosterone, decreased in corticosteroids, decrease in cortisol, increased in sex hormoones. HYPERtension and maculinization.
Term
What are the clinical features of Congenital adrenal hyperplasia due to 17 alpha0hydroxylase?
Definition
Increase in mineralcorticoids, decrease in both cortisol and sex hormones. HYPERtension, hypokalemia. XX: exrenall phenotypic females w/ normal internal sex organs, lacks secondary sex characteristics. XY: Decreased in DHT --> pseudohermaphroditism- variable ambigous genitalia
Term
A picture of a "sac of blood" adrenal glands is usually associated w/ what syndrome caused by N. meningitidis infection?
Definition
Watehouse-Friderichson syndrome
Term
What type of carcinoma likes to metastasize to the adrenal glands and can cause chornic adrenal insufficiecny?
Definition
Lung cancer
Term
What are the clinical features associated w/ chronic adrenal insufficiency?
Definition
HYPOtension, HYPOnatremia, HYPOvolemia, HYPErkalemia, weakness, HYPERpigmention (oral mucosa and skin), vomiting and diarrhea
Term
What is the origin of chromaffin cells of the adrenal medulla? What do they secrete?
Definition
Neural crest-derived; secrete catecholamines (EPI and NE)
Term
What are the clinical features of pheochromocytoma?
Definition
Episodic HTN, headache, palpitations, tachycardia and sweating
Term
What must be given to the patient BEFORE surgical removal of a pheochromocytoma and why?
Definition
Phenoxybenzamine (irreversible alpha blocker) in order to prevent a hypertensive crisis
Term
What is a common location of a pheochromocytoma that is located outside of the adrenal medulla?
Definition
Bladder wall or organ of Zuckerkandle at the IMA root.
Term
What are some syndromes that are associated w/ pheochromocytoma?
Definition
MEN2A, MEN2B, von Hippel-Lindau disease, and neurofibromatosis type I
Term
What is the most common pathogen associated w/ acute mastitis?
Definition
S. aureus
Term
What breast pathology is associated w/ a green-brown nipple discharge in a multiparous postmenopausal women?
Definition
Mammary duct ectasia. Inflammation w/ dilation (ectasia) of the subareolar ducts.
Term
What breast pathology presents as a subarelar mass w/ nipple retraction in smokers?
Definition
Periductal mastitis
Term
What are "blue domed cysts" on gross breast exam?
Definition
Benign fibrocystic change
Term
What has the highest risk of invasive breast carcinoma? Apocrine metaplasia or ductal hyperplasia and sclerosing adenosis or atypical hyperplasia?
Definition
Apocrine metaplasia: no increased risk. Ductal hyperplasia and sclerosing adenosis (calcified): 2x increased risk. Atypical hyperplasia: 5x increased risk.
Term
How can you distinguish a papillary breast carcinoma from an intraductal papilloma?
Definition
Papillary carcinomadoes NOT have the underlying myopepithelial cells (while the intraductal papilloma is lined by both the epithelial and myoepithelial cells.
Term
What two breast changes (pathologies) present with a bloody nipple discharge?
Definition
Intraductal papilloma and papillary carcinoma
Term
what is the most common tumor in premenopausal women? Does the woman with this have an increased risk for cancer?
Definition
Fibroadenoma; benign w/ no inreased risk of carcinoma
Term
What breast pathology has "leaf-life" projections?
Definition
Phyllodes tumor: overgrowth of the fibrous compoenent; commonly seen in postmenopausal women. Can be malignant.
Term
Describe the "comedo-type" of DIC in the breast?
Definition
Characterized by high-grade cells w/ necrosis and dystrophic calcification in the center of ducts
Term
What is the most common type on invasive carcinoma in the bresat?
Definition
Invasive ductal carcinoma
Term
What are the 4 subtypes of invasive ductal carcinoma?
Definition
1. Tubular carcinoma: good prognosis, 2. Mucinous carcinoma: good prognosis, seen in elderly women, 3. Medllary carcinoma: mimics fibroadenoma, increased incidence in BRCA1 carriers, good prognosis and 4. Inflammatory carcinom: looks like acute mastitis since it blocks the lymphatics; poor prognosis
Term
In lobular carcinoma in situ (LCIS) why are the cells dyscohesive?
Definition
They lack E-cadherin adhesion protein
Term
What breast pathology characteristically grow in a single file pattern and why do they grow that way??
Definition
Invasive locular carcinoma; they do not make ducts b/c they lack E-cadherin
Term
What are the most important predictive factors to treatment in breast cancer?
Definition
Estrogen receptor (ER), Progesterone receptor (PR), and HER2/neu amplication stutus
Term
What are the two most important single gene mutations associated w/ hereditary breast cancer?
Definition
BRCA1 and BRCA2
Term
What results in a "frog-like" appearance of the getus and maternal polyhydramnios?
Definition
Anencephaly b/c of disruption of the cranial end of the neural tube
Term
What presents as a massively dilated 4th ventricle w/ an absent cerebellum; often accompanied by hydrocephalus?
Definition
Dandy-walker malformation
Term
What is the difference b/w a meningocele and a meningomyelocele?
Definition
Meningocele: protrusion of meninges. Meningomyelocele: protrusion of meninges and spinal cord.
Term
What is Arnold-Chiar malfromation (type II)? What is it associated with?
Definition
Congenital extension of cerebellar tonsils through the foramen magnum --> obstruction of CSF can result in hydrocephalus. Associated w/ meningomyelocele and syringomyelia.
Term
What leads to sensory loss of pain and temp with sparring of fine touch and position in the upper extremties in a "cape-like" distribution?
Definition
Syringomyelia (cystic degeneration of the spinal cord) that usually occurs at C8-T1.
Term
What virus damages the anterior motor horn leading to lower motor neuron sign?
Definition
Poliovirus
Term
What Autosomal recessive disease presents as a "floppy baby" and in which death occurs w/in a few years after birth?
Definition
Werdnig-Hoffman disease
Term
What does a mutation in SOD2 gene lead to (think neurology)?
Definition
Amyotrophic lateral sclerosis (ALS); SOD1 (superoxide dismutase) is knocked out and that leads to free radical injury in neurons
Term
What is a degenerative disorder of both the upper and lower motor neurons of the corticospinal tract?
Definition
Amyotrophic lateral sclerosis (ALS)
Term
What causes Friedreich ataxia?
Definition
AR: due to expansion of GAA in the Frataxin gene.
Term
What is the role of the frataxin gene (in Friedreich Ataxia)?
Definition
Frataxin gene: essential for mitochondrial iron regulation; loss results in iron buildup w/ free radical damage
Term
What is a common cardiac abnormality in Friedreich Ataxia?
Definition
Associated w/ hypertrophic cardiomyopathy
Term
What is the most common cause of meningitis in a) neonates, b) children/teenagers, c) adults/elderly, d) non-vaccinated infants, e) viral cause in children
Definition
a) neonates: Group B streptococcus, E. coli, Listeria monocytogenes b) children/teenagers: N. meningitidis (it goes from the nasopharyn to the blood then to the meninges) c) adults/elderly: Streptococcus penumoniae d) non-vaccinated infants: H. influenzae type B e) viral cause in children:Coxsackievirus
Term
When doing a lumbar puncture, what are the layers that you go though and which layer do you NOT pierce?
Definition
Skin --> ligaments --> epidural space --> dura --> arachnoid. You DO NOT piece the PIA!
Term
What are the CSF findings for bacterial meningitis?
Definition
Neutrophils, decrease glucose
Term
What are the CSF findings for viral meningitis?
Definition
Lymphocytes w/ decreased glucose
Term
What are the CSF findings for fungal meningitis?
Definition
Lymphocytes w/ decreased glucose
Term
What is the a) origin and b) type of infat w/ a thrombotic stroke?
Definition
A) Due to rupture of an atherosclerotic plaque. B) Pale infarct at the periphery of the cortex
Term
What is the a) origin and b) type of infat w/ a embolic stroke?
Definition
A) Due to thromboemboli usually arising from the left side of the heart. B) Hemorrhagic infarct. Usually involves the middle cerebral artery
Term
What blood vessels does a lacunar stroke most commonly involved?
Definition
Lenticulostriate vessels
Term
What type of stroke woud a patient get if he has a lacunar stroke in the internal capsule?
Definition
Pure motor stroke
Term
What type of stroke woud a patient get if he has a lacunar stroke in the thalamus?
Definition
Pure sensory stroke
Term
What tpe of stroke is a "red neuron" associated with?
Definition
Ischemic stroke. Eosinophilic change in the cytoplasm of neurons early microscopic finid (12hrs post infarction).
Term
What type of necrosis does the brain undergo in an ischemic stroke? How many hours post infarction does this occur?
Definition
Coagulative necrosis; occurs 24hrs psot infarction
Term
What is the very end stage lesion of an ischemic stroke?
Definition
Formation of fluid-filled cystic space surrounded by gliosis. It is cystic b/c the area underwent liquifactive necrosis.
Term
What is the caue of the "worst headache" of my life? What is the most common cause? Where is the most common location of this bleed?
Definition
Subarachanoid hemorrhage. Berry aneursyms (lack a media layer-making it suspectible to rupture); most common location is at the anterior circle of Willis at the branch points of the anterior communicating artery.
Term
What syndromes are berry aneurysms associated with?
Definition
Marfan syndrome and autosomal dominant polycystic kidney disease
Term
What type of brain bleed has a len-shaped lesion on CT? What is the most common blood vessel involved? Where does the blood collect?
Definition
Epidural hematoma; Middle meningeal artery; Collection of blood b/w the dura and the skull.
Term
What type of brain bleed has a crescent-shaped lesion on CT? What is the most common blood vessel involved? Where does the blood collect?
Definition
Subdural hematoma; Rupture of a bridging vein. Collection of blood unerneath the dura.
Term
What occurs w/ a tonsilar herniation?
Definition
Displacement of the cerebellar tonsils into the foramen magnus --> compresion of the brain stem --> cardiopulmonary arrest
Term
What occurs w/ a subfalcine herniation?
Definition
Displacemtn of the cingulate gyrus under the falx cerebri --> compression of the anterior cerebral artery --> infarction
Term
What occurs w/ an uncal herniation?
Definition
Displacement of the temporal lobe uncus under the tentorium cerebelli --> get compression of CN III (get eye that moves down and out w/ a "blown" pupil), compression of posterior cerebral artery w/ infarction of posterior lobe (contralateral homonymous hemianopsia) and get rupture of the paramedian artery that leads to Duret (brainstem) hemorrage.
Term
What is Metachromatic leukodystrophy?
Definition
Due to deficiency of arylsulfatase (autosomal recessive). Myelin cannot be degraded and accumulates in the lysosomes of oligodendrocytes
Term
What is Krabbe disease?
Definition
Deficiency of galactocerebroside Beta-galactosidase (autosomal recessive); galactocerebroside accumulates in the macrophages
Term
What is adrenoleukodystrphy?
Definition
Imparied addition of conenzyme A to long-chain fatty acids (this is X linked). Accumulation of fatty acids damages adrneal gands and white matter of the brain.
Term
Multiple sclerosis
Definition
What diseaes involves autoimmune destruction of CNS myelin and oligodendrocytes?
Term
Given the following scenario, what is wrong w/ the patient? The patient is asked to look left: her left eye looks left but her right eye doesn't look left.
Definition
The patient has a right Medial longitiduinal fasciculus injury giving her internuclear opthalmoplegia (commonly associated w/ MS).
Term
Lumbar puncture shows: increased lymphocytes, increased immunoglobulins w/ oligoclonal IgG bands, and myelin basic protein. What can this be?
Definition
Multiple sclerosis
Term
What viral infection can lead to subacute sclerosing panencephalitis?
Definition
Measles virus
Term
What virus leads to Progresssive multifocal leukoencephalopathy? What does it present with?
Definition
JC virus infection of oligodendrocytes. Presents w/ rapidly progressive neurologic signs (visual loss, weakness, dementia) leading to death
Term
What classically presents as "locked in syndrome"? What usually leads to this?
Definition
Central pontine myelinolysis. Due to rapid IV correction of hypnatremia.
Term
What is the most common cause of dementia?
Definition
Alzheimer disease
Term
What APOE allele is associated w/ a) an increased risk of Alzheimer disease and b) with a decreased risk?
Definition
A) Increased risk: APOE epsilon 4 allele (accelerate the conversion of APP to A-beta-amyloid). B) Decreased risk: APOE epsilon 2 allele.
Term
What leads to early onset Alzheimer disease?
Definition
Familial cases: associated w/ presinilin 1 and presenilin 2 mutations. Down synddrome: commonly occurs by 40 yars of age b/c they have 3 copies of Ch21 which is what codeds for Amyloid precursor protein (APP) that gets converted to A-beta-amyloid and creates the neuritic plaques
Term
What creates the Neuritic (senile) plaques in Alzheimer's disease. What Chromosome is this coded on?
Definition
Amyloid precursor protein (APP) that gets converted to A-beta-amyloid and creates the neuritic (senile) plaques. The amyloid may deposit around vessels --> increasing the risk of intracranial hemorrhage. APP is coded on chromosome 21.
Term
What contributes to the dementia in Alzheimer's disease?
Definition
The neurofibrillary tangles which are intracellular aggregates of fibers composed of hyperphosphorylated tau which is a microtuble associated protein
Term
Is Acetylcholine increased or decreased in Alzheimer's disease?
Definition
It is decreased
Term
What does the brain look like on gross examination in Alzheimer's disease?
Definition
Cerebral atrophy w/ narrowing of the gyri, widening of the gyri, widening of the sulci, and dilation of the ventricle (hydrocephaly ex vacuole)
Term
What is the 2nd most common cause of dementia?
Definition
Vascular dementia b/c of multifocal infarction and injury due to HTN, atherosclerosis or vasculitis.
Term
What lobes does Pick's disease involve most?
Definition
Frontal and temporal cortex; spares the parietal and occipital lobes
Term
What would you see in Pick's disease?
Definition
Round aggregates of Tau protein (pick bodies) in neurons in the cortex.
Term
What neurons are lost in Parkinsons disease?
Definition
Loss of dopaminergic neurons in the substantia nigra of the basal ganglia. The nigrostriatal pathway of basal ganglia uses dopamine to initiate movement.
Term
What ilicit drug can result in drug -induced Parkinson's disease?
Definition
MPTP
Term
What are the clinical features of Parkinson disease?
Definition
TRAP. T: tremor which is pill rolling at rest and disappears w/ movement. R: rigidity (cogwheel rigidity in the extremities), A: Akinesia/bradykinesia (slowing of voluntary movement; expressionless face), P: Postural instability
Term
What is seen in histology of Parkinson disease?
Definition
Loss of pigmented neurons in the substantia nigra and round, eosinophilic inclusions of alpha-synuclein (Lewy bodies) in affected neurons.
Term
What is Lewy body dementia?
Definition
Parkinsonism w/ dementia and hallucinations. Get lewy body formation in the cortex of the brain.
Term
What disease is characterized by the degeneration of GABAergic neurons in the caudate nucleus of the basal ganglia?
Definition
Huntington disease
Term
What is the trinucelotide repeat seen in Huntington Disease?
Definition
CAG
Term
What is the presenation of Huntington disease?
Definition
Choreac that can progres to dementia and depression
Term
What is Creuzfeldt-Jakob disease (CJD)?
Definition
Rapidly progressivce (weeks to months) dementia w/ myoclonia. Get a spongiform cortex. Prior cause conversion of PrPc --> PrPsc (beta pleated sheet)
Term
What is the most common form of spongiform encephalopathy?
Definition
Creuzfeldt-Jakob disease (CJD)
Term
What presents as triad of urinary incontinenc, gait instability and dementia (wet, wobbly and wacky)?
Definition
Normal pressure hydrocephalus; due to the stretching of the coronar radiata
Term
When does the trinucleotide expansion of CAG occur leading to anticipation in Huntington disease?
Definition
It occurs during spermatogeneis.
Term
What is the cause of normal pressure hydrocephalus?
Definition
There is too much CSF around b/c there is decreased absorption of CSF into the arachnoid granulation
Term
What are the types of complexes seen on EKG in a patient w/ Creuzfeldt-Jakob disease (CJD)?
Definition
Spike waves complexes
Term
Most adult tumors are? (supratentorial or infratentorial)
Definition
Supratentorial
Term
Most child tumors are? (supratentorial or infratentorial)
Definition
Infratentorial
Term
What is the most common primary malignant CNS tumor in adults? In children?
Definition
Adults: glioblastoma multiforme (GBM). Children: pilocytic astrocytoma
Term
What CNS tumor characteristically crosses the corpus callosum (butterfly lesion) and has pseudopalisading cells?
Definition
Gliobastoma multiforme
Term
What is the intermediate filament that is present in Glioblastoma multiforme?
Definition
GFAP
Term
What is the most common benign CNS tumor in adults?
Definition
Meningioma
Term
Which CNS tumor shows a whorled patten on histology and psammoma bodies?
Definition
Meningioma
Term
What resectable CNS tumor is S-100+
Definition
Schwannoma
Term
What syndrome is associated w/ bilateral vestibular schwannomas?
Definition
Neurofibromatosis type 2
Term
What CNS tumor shows up as a calcified tumor that has a "fried-egg" apperance to it?
Definition
Oligodendroglioma
Term
Which CNS tumor has a chicken-wire capillary pattern to it?
Definition
Oligodendroglioma
Term
Which CNS tumor has Rosenthal fibers, is GFAP positive and has a relatively good prognosis to it?
Definition
Pilocytic astrocytoma
Term
Which CNS tumor derives from neuroectoderm and has Homer-Wright rosettes?
Definition
Medulloblastoma
Term
Which CNS tumor is characterized by perivascular pseudorosettes?
Definition
Ependymoma
Term
Which CNS tumor arises from the Ratheke's pouch?
Definition
Craniopharyngioma
Term
Which CNS tumor can lead to bitemporal hemianopsia in a child or young adult?
Definition
Craniopharyngioma
Term
What is the mutation that leads to achondroplasia? How does it work?
Definition
Overexpression of FGFr3 inhibits chondrocyte proliferation
Term
Why do patients w/ achondroplasia have short extremities but normal sized head and chest?
Definition
They have poor endochondral bone formation (formation of a cartilage matrix -> replaced by bone. They have normal intramembranous bone formation (formation of bone w/o pre-existing cartilage matrix).
Term
A patient present w/ multiple fractures, blue sclero and hearing loss. What does he have?
Definition
Osteogenesis imperfectat; most commonly due to autosomal dominant defect in collagen type I
Term
Why is the sclera blue in osteogenesis imperfecta?
Definition
Thnning of sclear collagen reveals underlying choridal veins
Term
Osteopetrosis: what does a carbonic anhydrase II mutation lead to?
Definition
Leads to loss of the acidic environment required for bone resorption
Term
What are the clinical features of osteopetrosis?
Definition
Bone fracutres, anemia, thrombocytopneia and leukopenia w/ extramedullary hematopoiesis, vision and hearing impairment, hydrocephalus, renal tubular acidosis
Term
What is the treatment of osteopetrosis?
Definition
Bone marrow transplant- osteoclast are macrophages (derived from monocytes), so doing a BM transplant would reseed the bones w/ new osteoclast
Term
What does decreased Vit D in adults vs children called?
Definition
Adults: osteomalacia. Children: rickets
Term
What are some clinical features of Vit D deficiency (rickets) in children?
Definition
Pigeon-breat deformity, Frontal bossing, rachitic rosary, bowing of the legs. All of these characteristics are due to the deposition of osteoid
Term
What are the laboratory findings in osteomalacia?
Definition
Decreased serum calcium, decreased serum phosphate, increased PTH and increased alkaline phosphatase
Term
What is osteoporosis?
Definition
Reduction in trabecular bone mass that results in porous bone w/ an increased risk for fracture.
Term
What are the lab findings in osteoprosis?
Definition
Everything is normal.
Term
Why does Paget disease of bone occurs?
Definition
It is due to imbalance of osteoclast and osteoblast function.
Term
What are the 3 stages of Paget's disease of bone? What is the end result? What does the biopsy reveal?
Definition
1. Osteoclastic, 2. Mixed osteoblastic and osteoclastic and 3. Osteoblastic. The end result is thick, sclerotic bone that fractures easily. Biopsy reveals a mosaic pattern of lamellar bone.
Term
What are the clinical features of Paget's disease of bone?
Definition
Bone pain, increasing hat size, hearing loss, lion0like facies, isolated elevated alkaline phosphatase
Term
What are the treatments of Paget's disease of bone?
Definition
Calcitonin and bisphosphonates.
Term
What are the complications of Paget's disease of bone. This is HIGH YIELD!
Definition
High output cardiac failure (due to the formation of AV shunts in bone), osteosarcoma
Term
In osteomyelitis, in what part of the bone does the bacteria seed in adults vs in kids? (choices are metaphysis and epiphysis)
Definition
Child: seens in metaphysis. Adult: seeds in epiphysis.
Term
What are the most common causes of osteomyelitis in: a) overall, b) sexually active young adults, c) Sickle cell disease, d) Diabetics or IV drug users, e) Associated w/ cat/dog bite scratches, f) Usually involving the vertebrae.
Definition
a) Overall: S. aureus b) sexually active young adults: N. gonorrhoeae c) Sickle cell disease: Salmonella d) Diabetics or IV drug users: Pseudomonas e) Associated w/ cat/dog bite scratches: Pasteurealla f) Usually involving the vertebrae: Mycobacteriumm tuberculosis (Pott Disease)
Term
Where does an osteoma most commonly occur? What syndrome is it associated with?
Definition
It arises on the surface of facial bones. It is associated w/ Gardner syndrome
Term
What is the classic presentation of an osteoid osteoma?
Definition
Presents as bone pain that resolves w/ ASPIRIN!
Term
What type of bone tumor presents as bone pain that does NOT respond to aspirin?
Definition
Osteoblastoma
Term
What type of bone tumor is present on imaging as a bony mass (<2cm) w/ a radiolucent core?
Definition
Osteoid osteoma
Term
What is the most common benign tumor of bone?
Definition
Osteochondroma
Term
What part of the bone gives rise to an osteochondroma?
Definition
It arises from a lateral projection of the growth plate (metaphysis); bone is continous w/ the marrow space.
Term
What part of the bone gives rise to an osteosarcoma (osteogenic sarcoma)?
Definition
Arises in the metaphysis of long bones, usually the distal femur or proximal tibia (region of the knee)
Term
What part of the bone gives rise to Giant cell tumor (osteoclastoma)?
Definition
Arises in the epiphysis of long bones, usualy the distal femur or proximal tibia.
Term
Which bone tumor has a "soap bubble" appearance to it?
Definition
Giant cell tumor (osteoclastoma)
Term
Which bone tumor has a Codman's triangle or sunburst pattern to it on X-ray?
Definition
Osteosarcoma (osteogenic sarcoma)
Term
Which bone tumor is assoacited w/ the (11:22) translocation?
Definition
Ewing sarcoma
Term
Which bone tumor has an "onion-skin" appearance to it?
Definition
Ewing sarcoma
Term
What type of cell does Ewing sarcoma arise from?
Definition
Neuroectoderm
Term
What is the most common benign bone tumor?
Definition
Osteochondroma
Term
What cartilage tumor a) arises in the medulla of the small bones of the hands/feet? B) arises in medulla of the pelvis or central skeleton? C) Which ones of these is malignant?
Definition
A) Chondroma. B) Chondrosarcoma C) Chondrosarcoma is MALIGNANT
Term
Which type of metastatic tumor produces osteoblastic lesions as opposed to most metastatic tumors that produce osteolytic lesions?
Definition
Prostate carcinoma
Term
What type of collagen is found in cartilage?
Definition
Type II
Term
What type of disease has "joint mice", osteophytes in the DIP (Heberden nodes) and PIP (Bouchard nodes)?
Definition
Degenerative joint disease (Osteoarthritis)
Term
What is the HLA association of Rheumatoid arthritis?
Definition
HLA-DR4
Term
What type of arthritis presents w/ a) joint stiff in the morning and it worsens during the day as opposed to b) morning stiff that improves w/ activity?
Definition
a) Osteoarthritis. B) Rheumatoid arthritis
Term
The hallmark of disease "x" is synovitis leading to formation of pannus (inflammed granulation) that leads to destruction of cartilaage and ankylosis of the joint. Name disease "x"
Definition
X = Rheumatoid arthritis
Term
What are the classic joints involved in Rheumatoid arthritis? Which joints are classicaly spared?
Definition
Joint involved: PIP of the giners (swan-neck deformity), wrists (ulnar deviation), elbwos, ankles, and knees. Spared: DIP
Term
What is Rheumatoid factor? What is it a marker of?
Definition
IgM autoantibody against Fc portion of IgG; marker of tissue damage and disease activity
Term
Name seronegative spondyloarthropathies
Definition
Ankylosing spondyloarthritis, Reiter syndrome, Psoriatic arthritis
Term
"Can't see, can't pee and can't climb a tree" refers to what type of seronegative spondyloarthropathy?
Definition
Reiter syndrome. Characterized by a triad of arthritis, urethritis and conjunctivitis.
Term
What is an extra-articular manifestation of ankylosing spondyloarthritis?
Definition
Uveitis and aortitis (that can lead to aortic regurgitation)
Term
Which HLA are seronegative spondyloarthropathy associated with?
Definition
HLA-B27
Term
What are secondary causes of gout?
Definition
Leukemia and myeloproliferative disorders, Lesch-Nyhan syndrome, Renal insufficiency
Term
What is the deficiency that leads to Lesch-Nyhan syndrome?
Definition
X linked deficiency of hypoxantine-guanine-phosphoribosyltransferase (HGPRT)
Term
What activates the neutrophils in gout?
Definition
The monosodium urate (MSU) crystals
Term
What is podagra?
Definition
Exquisitely painful arthritis of the great toe
Term
What are triggers of gout?
Definition
Alcohol or consumption of meat
Term
What can chronic gout lead to?
Definition
Development of tophi (white, chalky aggregates of uric acid cyrstals w/ fibrosis and giant cell reaction in the soft tissue and joints) and renal failure (urate crystals deposit in the kidney tubules --> urate nephropathy)
Term
What are the laborary findings of gout?
Definition
Hyperuricemia. Crystals are needle-sh.aped and have negative birefringence under polarized light (they're YELLOW). Crystals are yellow when parallel and blue when perpendicular to the ligt.
Term
What are the laboratory findings in pseudogout?
Definition
Deposition of calcium pyrophosphate dihydrate (CPPD). Rhomboid shaped crystals w/ weakly positive birefringence under poralized light. Crystals are yellow when perpendicular light and blue when parallel to the light.
Term
If a patient presents w/ bilateral proximal muslce weaknes (he can't comb his hair), rash of the upper eyelids, malar rash, red papules on the elbows/knuckles/knees (Grotton lesion), what may he have? What other etiology should you follow up on?
Definition
He most likely has dermatomyositis. Most cases are associated w/ an underlying gastric carcinoma.
Term
What are the laboratory findings for dermatomyositis?
Definition
Increased creatine kinase, positive ANA and anti-Jo-1 antibody, perimysial inflammation (CD4+T cells) w/ perifasciular atrophy on biopsy (high yield).
Term
What are the laboratory findings of polymyositis?
Definition
Endomysial inflammation (CD8+T cells) w/ necrotic muscle fibers seen on biopsy
Term
A patient prsents w/ calf pseudohytrophy and increased creatinine kinase. His uncle presented with similar symptoms but died of cardiac failure at a young age. What does this patient have?
Definition
Duchenne muscular dystrophy. Deletion of dystrophin.
Term
A woman presents w/ ptosis, diplopia, muscle weakness that worsens w/ use and improves with rest . What does most likely have? What causes this?
Definition
Myasthenia gravis; autoantibodies against the postsynaptic acetylcholine receptor at the neuromuscular junction
Term
What is Myasthenia gravis associated with? (HIGH YIELD)
Definition
Thymic hyperplasia or thymoma
Term
A patient presents w/ proximal weakness that improves w/ use. He is also a smoker and was diagnosed with Small cell lung carcinoma. What does he have?
Definition
Lamber eaton syndrome
Term
What is the cause of lambert eaton syndrome?
Definition
Antibodies against presynaptic calcium channels of the neuromuscular junction --> leads to impaired acetylcholine release
Term
What is the most common bening soft tissue tumor in adults? What is the most common malignant soft tissue tumor in adults?
Definition
Lipoma; liposarcoma
Term
What is the characteristic cell in a liposarcoma?
Definition
Lipoblast
Term
What is the most common malight soft tissue tumor in children?
Definition
Rhabdomyosarcoma
Term
What cell is demin positive?
Definition
Rhabdomyosarcoma
Term
What is the classic site of presentation of a Rhabdomyosarcoma in a young girl?
Definition
The vagina- it's the grape like clusters that appear in the vaginal opening. They're demin positive and have straitions b/c they're of muscle cell origin.
Term
What is the regenerative (stem cell) layer of the epidermis?
Definition
Stratum basalis
Term
What is the pathogen that is associated w/ acne vulgaris? How does it cause acne?
Definition
Propionibacterium acnes infection produces lipases that break down sebum, releasing proinflammatory fatty acids
Term
What is a well-circumscribed, salmon-colored plaque w/ silvery scale, usually on extensor surfaces and the scalp?
Definition
Psoriasis
Term
Why does psoriasis occur; what cell in the epidermis is involved?
Definition
Possible autoimmune etiology; associated w/ HLA-C. Excessive keratinocyte proliferation
Term
What derm disease is associated w/ Auspitz sign and Munro microabscesses.
Definition
Psoriasis. Auspitz sign: bleeding when the scale is picked off b/c of thinning of the epidermias above elongated dermal papilla. Munro microabscesses: collections of neutrophils in the stratum corneum
Term
What is a pruritic, planar, polygonal, purple plaque?
Definition
Lichen planus
Term
What type of derm disease has histology that shows inflammation of the dermal-epidermal junction w/ a "saw-tooth" appearance?
Definition
Lichen planus
Term
What virus is associated w/ Lichen planus?
Definition
Lichen planus
Term
What derm disease is associated w/ autoimmune destruction of desmosomes b/w keratinocytes?
Definition
Pemphigus vulgaris
Term
What is the autoAb in pemphigus vulgaris?
Definition
IgG antibody against desmoglein (type II hypersensitivity)
Term
What derm pathology shows a "tombstone" appearance on hsitology?
Definition
Pemphigus vulgaris
Term
What derm pathologies show "fish net" patterns on immunofluoresence?
Definition
Pemphigus vulgaris and Bullous pemphigoid.
Term
Which derm pathology (pemphigus vulgaris or bullour pemphigoid) is most likely to involve the oral mucosa?
Definition
Pemphigus vulgaris
Term
Which derm pathology is due to IgG antidoby against basement membrane collagen?
Definition
Bullous pemphigoid
Term
Which derm pathology (pemphigus vulgaris or bullour pemphigoid) has a tense and which has a flaccid bullous?
Definition
Tense: Bullous pemphigoid. Flaccid: Pemphigus vulgaris
Term
What is dermattitis herpatiformis? What is is associated with?
Definition
Autoimmune deposition of IgA at the tips of dermal papilae. Strong association w/ CELIAC disease. This resolves w/ a glutten free diet.
Term
What are the most common pathogens associated w/ Erythema multiforme?
Definition
#1 HSV infection, #2 Mycoplasma infection
Term
What is Leser-Trelat sign? What is it associated with?
Definition
Sudden onset of multiple seborrheic keratoses and suggests an underlying carcinoma of the GI tract.
Term
What is acanthosis nigracans assocaited with?
Definition
Insulin resistance (Type II DM can get it) and gastric carcinoma
Term
What derm pathology shows peripheral palisading on histology?
Definition
Basal cell carcinoma
Term
What derm pathology presents as an elevated nodule w/a central, ulcerated crater surrounded by telangiectastit cells (pink, pearl-like papule)?
Definition
Basal cell carcinoma
Term
What derm path (BCC or SCC) most likely presents on a) upper lip or b) lower lip?
Definition
A) BCC, B) SCC
Term
What is the most common skin cancer that arises in immunosuppressive therapy?
Definition
Squamous cell carcinoma
Term
What is a derm path that is a cup-shaped tumor filled w/ keratin debris that arises rapidly and regresses spontaneously?
Definition
Keratoacanthoma
Term
Where is melanin made in the melanocyte? What is the precursor to melanin?
Definition
Made in the melanosomes using tyrosine as a precursor molecule --> melanosomes get passed to keratinocytes.
Term
Where are melanocytes derived from?
Definition
Neural crest cells
Term
What is the disease associated w/ AUTOimmune destruction of melanocytes?
Definition
Vitiligo
Term
What is the disease that is assocaited w/ the congenital lack of pigmentation? What cancers are these patients at increased risk for?
Definition
Albinism; SCC, BCC and melanoma
Term
Why does a freckle (ephelis) look tan-dark brown and darker when exposed to sunlight?
Definition
It has an increased number of melanosmoes (melanocytes are NOT increased).
Term
What is the most common subtype of melanoma?
Definition
Superficial spreading melonomoa
Term
What is the most common cause of death from skin cancer?
Definition
Melanoma
Term
What determines the risk of metastasis in melanoma?
Definition
Depth of extension (Breslow thickness): most important prognosit factor in predicting metastasis.
Term
What pathogens cause impetigo?
Definition
S. aures and S. pyogenes
Term
What toxins of S. aureus cause Staphylococcal scalded skin syndrome?
Definition
Exfoliative A and B toxins -> results in epidermolysis of the stratum granulosum
Term
What causes Molluscum contagiosum? What is the name of the cytopasmic inclusion?
Definition
Poxvirus; cytoplasmic inclusions: molluscum bodies
Term
What is a frim, pink. UMBILICATED papule in an immunocompromised person? What is the pathogen that causes this?
Definition
Molluscum contagiosum; poxvirus
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