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
|
|
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
|
|
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
|
|
Term
| Which part of the spleen gets trapped w/ TRAP + cells in Hairy Cell Leukemia that leads to splenomegaly? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
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
|
|
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
|
|
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
|
|
Term
| What cardiac marker is the good standard for detecting an MI? |
|
Definition
|
|
Term
| In the first 6hrs of an MI, what should you do in terms of testing? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
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
|
|
Term
|
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
|
|
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
|
|
Term
| What type of congenital cardiac defect is associated w/ a "boot shaped" heart on X-ray? |
|
Definition
|
|
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
|
|
Term
| What type of weird vavle is the adult form of Coarction of the aorta associated with? |
|
Definition
|
|
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
|
|
Term
| What type of murmur am I? Early, high pitched blowing diastolic murmur |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
Term
| What type of murmur am I? Late systolic crescendon murmur w/ midsystolic click |
|
Definition
|
|
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
|
|
Term
| What organism should you suspect if the patient has endocarditis w/ negative blood cultures? |
|
Definition
|
|
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
|
|
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
|
|
Term
| Most common cause of laryngotracheobronchitis (CROUP)? |
|
Definition
| Parinfluenza virus (which is a paramyxovirus) |
|
|
Term
| What causes laryngeal papilloma? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
Term
| What is the most common cuase of interstitial (atypical) pneumonia in patients w/ post transplant immunosuppressive therapy? |
|
Definition
|
|
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
|
|
Term
| What type of granuloma is seen in TB? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
Term
| In which disease will you see a "honeycomb" lung? |
|
Definition
| Idiopathic pulmonary fibrosis (restrictive disease) |
|
|
Term
|
Definition
| Rheumatoid arthritis that is associated w/ Coal worker's pneumoconiosis |
|
|
Term
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
Term
| What is more common to the lung: primary tumor or metastasis? |
|
Definition
|
|
Term
| Where does lung cancer love to metastasize? |
|
Definition
|
|
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
|
|
Term
| What type of pneumothorax shifts the trachea to the opposite side of the collapse? |
|
Definition
|
|
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
|
|
Term
| Where does HSV-1 lay dormant that will lead to a "cold sore"? |
|
Definition
|
|
Term
| What are the risk factors for squamous cell carcinoma of the oral mucosa? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
Term
|
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
|
|
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
|
|
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
|
|
Term
| What part of the stomach does chronic autoimmune gastritis involve? |
|
Definition
|
|
Term
| What is the most common site for chronic H. pylori gastritis? |
|
Definition
|
|
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
|
|
Term
| What type of ulcer am I? Pain is DECREAED w/ meals and there is weight gain |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
Term
| What are clinical features of duodenal atresia? |
|
Definition
| Polyhydramnios, "double bubble" sign , bilious vomiting |
|
|
Term
|
Definition
|
|
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
|
|
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
|
|
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
|
|
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
|
|
Term
| What is the most common site of Carcinoid tumor in the GI tract? |
|
Definition
|
|
Term
| What does the carcinoid tumor? |
|
Definition
|
|
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
|
|
Term
| What has a "string-sign" on imaging? |
|
Definition
|
|
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
|
|
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
|
|
Term
| What syndrome is Hirschsprung disease associated with? |
|
Definition
|
|
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
|
|
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
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
Term
| Which two hepatitis viruses are transmitted fecal-oral? |
|
Definition
|
|
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
|
|
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
|
Definition
| Damaged intermediate filaments (cytokeratin filaments) |
|
|
Term
| What are the elevated liver enzymes in alcohol related liver disease? |
|
Definition
|
|
Term
| What liver enzyme is preferentially increased when there is alcohol based damage? |
|
Definition
|
|
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
|
|
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
|
|
Term
| What am I? Increase urinary coppe, decrease serum ceruloplasmia, and increased copper on liver biopsy |
|
Definition
|
|
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
|
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
|
|
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
|
|
Term
| What is associated w/ brown, granlar casts seen in the urine, Elevated BUN and creatinine, hyperkalemia w/ metabolic acidosis? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
Term
| What is PAS=, Keratin-, and S100+? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
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
|
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
|
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
|
|
Term
| What is the most common type of germ cell tumor in females? |
|
Definition
|
|
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
|
|
Term
| What type of germ cell tumor presents as a mass w/ clear cytoplasm and central nuclei? |
|
Definition
|
|
Term
| What is the male counterpart of a female dysgerminoma? |
|
Definition
|
|
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
|
|
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
|
|
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
|
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
|
|
Term
| In preeclampsia, what type of necrosis will you see in the vessels of the placenta? |
|
Definition
|
|
Term
|
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
|
|
Term
| What is a "snowstorm" apperance on ultrasound? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
Term
| What is the mechanism of biopsing the thyroid? |
|
Definition
|
|
Term
| What type of thyroid neoplasm involes a benign proliferation of follicles surrounded by a FIBROUS capsule? |
|
Definition
|
|
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
|
|
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
|
|
Term
| What is the gene mutation that is associatd w/ medullary carcinoma seen in MEN 2A and 2B? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
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
|
|
Term
| What Autosomal recessive disease presents as a "floppy baby" and in which death occurs w/in a few years after birth? |
|
Definition
|
|
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
|
|
Term
| What type of stroke woud a patient get if he has a lacunar stroke in the internal capsule? |
|
Definition
|
|
Term
| What type of stroke woud a patient get if he has a lacunar stroke in the thalamus? |
|
Definition
|
|
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
|
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
|
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
|
|
Term
| What viral infection can lead to subacute sclerosing panencephalitis? |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
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
|
|
Term
| What is the trinucelotide repeat seen in Huntington Disease? |
|
Definition
|
|
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
|
|
Term
| Most adult tumors are? (supratentorial or infratentorial) |
|
Definition
|
|
Term
| Most child tumors are? (supratentorial or infratentorial) |
|
Definition
|
|
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
|
|
Term
| What is the intermediate filament that is present in Glioblastoma multiforme? |
|
Definition
|
|
Term
| What is the most common benign CNS tumor in adults? |
|
Definition
|
|
Term
| Which CNS tumor shows a whorled patten on histology and psammoma bodies? |
|
Definition
|
|
Term
| What resectable CNS tumor is S-100+ |
|
Definition
|
|
Term
| What syndrome is associated w/ bilateral vestibular schwannomas? |
|
Definition
|
|
Term
| What CNS tumor shows up as a calcified tumor that has a "fried-egg" apperance to it? |
|
Definition
|
|
Term
| Which CNS tumor has a chicken-wire capillary pattern to it? |
|
Definition
|
|
Term
| Which CNS tumor has Rosenthal fibers, is GFAP positive and has a relatively good prognosis to it? |
|
Definition
|
|
Term
| Which CNS tumor derives from neuroectoderm and has Homer-Wright rosettes? |
|
Definition
|
|
Term
| Which CNS tumor is characterized by perivascular pseudorosettes? |
|
Definition
|
|
Term
| Which CNS tumor arises from the Ratheke's pouch? |
|
Definition
|
|
Term
| Which CNS tumor can lead to bitemporal hemianopsia in a child or young adult? |
|
Definition
|
|
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
|
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
|
|
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. |
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Term
| What are the clinical features of Paget's disease of bone? |
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Definition
| Bone pain, increasing hat size, hearing loss, lion0like facies, isolated elevated alkaline phosphatase |
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Term
| What are the treatments of Paget's disease of bone? |
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Definition
| Calcitonin and bisphosphonates. |
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Term
| What are the complications of Paget's disease of bone. This is HIGH YIELD! |
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Definition
| High output cardiac failure (due to the formation of AV shunts in bone), osteosarcoma |
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Term
| In osteomyelitis, in what part of the bone does the bacteria seed in adults vs in kids? (choices are metaphysis and epiphysis) |
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Definition
| Child: seens in metaphysis. Adult: seeds in epiphysis. |
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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. |
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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) |
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Term
| Where does an osteoma most commonly occur? What syndrome is it associated with? |
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Definition
| It arises on the surface of facial bones. It is associated w/ Gardner syndrome |
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Term
| What is the classic presentation of an osteoid osteoma? |
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Definition
| Presents as bone pain that resolves w/ ASPIRIN! |
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Term
| What type of bone tumor presents as bone pain that does NOT respond to aspirin? |
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Definition
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Term
| What type of bone tumor is present on imaging as a bony mass (<2cm) w/ a radiolucent core? |
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Definition
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Term
| What is the most common benign tumor of bone? |
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Definition
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Term
| What part of the bone gives rise to an osteochondroma? |
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Definition
| It arises from a lateral projection of the growth plate (metaphysis); bone is continous w/ the marrow space. |
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Term
| What part of the bone gives rise to an osteosarcoma (osteogenic sarcoma)? |
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Definition
| Arises in the metaphysis of long bones, usually the distal femur or proximal tibia (region of the knee) |
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Term
| What part of the bone gives rise to Giant cell tumor (osteoclastoma)? |
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Definition
| Arises in the epiphysis of long bones, usualy the distal femur or proximal tibia. |
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Term
| Which bone tumor has a "soap bubble" appearance to it? |
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Definition
| Giant cell tumor (osteoclastoma) |
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Term
| Which bone tumor has a Codman's triangle or sunburst pattern to it on X-ray? |
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Definition
| Osteosarcoma (osteogenic sarcoma) |
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Term
| Which bone tumor is assoacited w/ the (11:22) translocation? |
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Definition
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Term
| Which bone tumor has an "onion-skin" appearance to it? |
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Definition
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Term
| What type of cell does Ewing sarcoma arise from? |
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Definition
|
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Term
| What is the most common benign bone tumor? |
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Definition
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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? |
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Definition
| A) Chondroma. B) Chondrosarcoma C) Chondrosarcoma is MALIGNANT |
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Term
| Which type of metastatic tumor produces osteoblastic lesions as opposed to most metastatic tumors that produce osteolytic lesions? |
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Definition
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Term
| What type of collagen is found in cartilage? |
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Definition
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Term
| What type of disease has "joint mice", osteophytes in the DIP (Heberden nodes) and PIP (Bouchard nodes)? |
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Definition
| Degenerative joint disease (Osteoarthritis) |
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Term
| What is the HLA association of Rheumatoid arthritis? |
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Definition
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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? |
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Definition
| a) Osteoarthritis. B) Rheumatoid arthritis |
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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" |
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Definition
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Term
| What are the classic joints involved in Rheumatoid arthritis? Which joints are classicaly spared? |
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Definition
| Joint involved: PIP of the giners (swan-neck deformity), wrists (ulnar deviation), elbwos, ankles, and knees. Spared: DIP |
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Term
| What is Rheumatoid factor? What is it a marker of? |
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Definition
| IgM autoantibody against Fc portion of IgG; marker of tissue damage and disease activity |
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Term
| Name seronegative spondyloarthropathies |
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Definition
| Ankylosing spondyloarthritis, Reiter syndrome, Psoriatic arthritis |
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Term
| "Can't see, can't pee and can't climb a tree" refers to what type of seronegative spondyloarthropathy? |
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Definition
| Reiter syndrome. Characterized by a triad of arthritis, urethritis and conjunctivitis. |
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Term
| What is an extra-articular manifestation of ankylosing spondyloarthritis? |
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Definition
| Uveitis and aortitis (that can lead to aortic regurgitation) |
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Term
| Which HLA are seronegative spondyloarthropathy associated with? |
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Definition
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Term
| What are secondary causes of gout? |
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Definition
| Leukemia and myeloproliferative disorders, Lesch-Nyhan syndrome, Renal insufficiency |
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Term
| What is the deficiency that leads to Lesch-Nyhan syndrome? |
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Definition
| X linked deficiency of hypoxantine-guanine-phosphoribosyltransferase (HGPRT) |
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Term
| What activates the neutrophils in gout? |
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Definition
| The monosodium urate (MSU) crystals |
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Term
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Definition
| Exquisitely painful arthritis of the great toe |
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Term
| What are triggers of gout? |
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Definition
| Alcohol or consumption of meat |
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Term
| What can chronic gout lead to? |
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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) |
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Term
| What are the laborary findings of gout? |
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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. |
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Term
| What are the laboratory findings in pseudogout? |
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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. |
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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? |
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Definition
| He most likely has dermatomyositis. Most cases are associated w/ an underlying gastric carcinoma. |
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Term
| What are the laboratory findings for dermatomyositis? |
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Definition
| Increased creatine kinase, positive ANA and anti-Jo-1 antibody, perimysial inflammation (CD4+T cells) w/ perifasciular atrophy on biopsy (high yield). |
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Term
| What are the laboratory findings of polymyositis? |
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Definition
| Endomysial inflammation (CD8+T cells) w/ necrotic muscle fibers seen on biopsy |
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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? |
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Definition
| Duchenne muscular dystrophy. Deletion of dystrophin. |
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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? |
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Definition
| Myasthenia gravis; autoantibodies against the postsynaptic acetylcholine receptor at the neuromuscular junction |
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Term
| What is Myasthenia gravis associated with? (HIGH YIELD) |
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Definition
| Thymic hyperplasia or thymoma |
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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? |
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Definition
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Term
| What is the cause of lambert eaton syndrome? |
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Definition
| Antibodies against presynaptic calcium channels of the neuromuscular junction --> leads to impaired acetylcholine release |
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Term
| What is the most common bening soft tissue tumor in adults? What is the most common malignant soft tissue tumor in adults? |
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Definition
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Term
| What is the characteristic cell in a liposarcoma? |
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Definition
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Term
| What is the most common malight soft tissue tumor in children? |
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Definition
|
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Term
| What cell is demin positive? |
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Definition
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Term
| What is the classic site of presentation of a Rhabdomyosarcoma in a young girl? |
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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. |
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Term
| What is the regenerative (stem cell) layer of the epidermis? |
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Definition
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Term
| What is the pathogen that is associated w/ acne vulgaris? How does it cause acne? |
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Definition
| Propionibacterium acnes infection produces lipases that break down sebum, releasing proinflammatory fatty acids |
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Term
| What is a well-circumscribed, salmon-colored plaque w/ silvery scale, usually on extensor surfaces and the scalp? |
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Definition
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Term
| Why does psoriasis occur; what cell in the epidermis is involved? |
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Definition
| Possible autoimmune etiology; associated w/ HLA-C. Excessive keratinocyte proliferation |
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Term
| What derm disease is associated w/ Auspitz sign and Munro microabscesses. |
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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 |
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Term
| What is a pruritic, planar, polygonal, purple plaque? |
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Definition
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Term
| What type of derm disease has histology that shows inflammation of the dermal-epidermal junction w/ a "saw-tooth" appearance? |
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Definition
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|
Term
| What virus is associated w/ Lichen planus? |
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Definition
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Term
| What derm disease is associated w/ autoimmune destruction of desmosomes b/w keratinocytes? |
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Definition
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Term
| What is the autoAb in pemphigus vulgaris? |
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Definition
| IgG antibody against desmoglein (type II hypersensitivity) |
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Term
| What derm pathology shows a "tombstone" appearance on hsitology? |
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Definition
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Term
| What derm pathologies show "fish net" patterns on immunofluoresence? |
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Definition
| Pemphigus vulgaris and Bullous pemphigoid. |
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Term
| Which derm pathology (pemphigus vulgaris or bullour pemphigoid) is most likely to involve the oral mucosa? |
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Definition
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Term
| Which derm pathology is due to IgG antidoby against basement membrane collagen? |
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Definition
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Term
| Which derm pathology (pemphigus vulgaris or bullour pemphigoid) has a tense and which has a flaccid bullous? |
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Definition
| Tense: Bullous pemphigoid. Flaccid: Pemphigus vulgaris |
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Term
| What is dermattitis herpatiformis? What is is associated with? |
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Definition
| Autoimmune deposition of IgA at the tips of dermal papilae. Strong association w/ CELIAC disease. This resolves w/ a glutten free diet. |
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Term
| What are the most common pathogens associated w/ Erythema multiforme? |
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Definition
| #1 HSV infection, #2 Mycoplasma infection |
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Term
| What is Leser-Trelat sign? What is it associated with? |
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Definition
| Sudden onset of multiple seborrheic keratoses and suggests an underlying carcinoma of the GI tract. |
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Term
| What is acanthosis nigracans assocaited with? |
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Definition
| Insulin resistance (Type II DM can get it) and gastric carcinoma |
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Term
| What derm pathology shows peripheral palisading on histology? |
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Definition
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Term
| What derm pathology presents as an elevated nodule w/a central, ulcerated crater surrounded by telangiectastit cells (pink, pearl-like papule)? |
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Definition
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Term
| What derm path (BCC or SCC) most likely presents on a) upper lip or b) lower lip? |
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Definition
|
|
Term
| What is the most common skin cancer that arises in immunosuppressive therapy? |
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Definition
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Term
| What is a derm path that is a cup-shaped tumor filled w/ keratin debris that arises rapidly and regresses spontaneously? |
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Definition
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Term
| Where is melanin made in the melanocyte? What is the precursor to melanin? |
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Definition
| Made in the melanosomes using tyrosine as a precursor molecule --> melanosomes get passed to keratinocytes. |
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Term
| Where are melanocytes derived from? |
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Definition
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Term
| What is the disease associated w/ AUTOimmune destruction of melanocytes? |
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Definition
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Term
| What is the disease that is assocaited w/ the congenital lack of pigmentation? What cancers are these patients at increased risk for? |
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Definition
| Albinism; SCC, BCC and melanoma |
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Term
| Why does a freckle (ephelis) look tan-dark brown and darker when exposed to sunlight? |
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Definition
| It has an increased number of melanosmoes (melanocytes are NOT increased). |
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Term
| What is the most common subtype of melanoma? |
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Definition
| Superficial spreading melonomoa |
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|
Term
| What is the most common cause of death from skin cancer? |
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Definition
|
|
Term
| What determines the risk of metastasis in melanoma? |
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Definition
| Depth of extension (Breslow thickness): most important prognosit factor in predicting metastasis. |
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Term
| What pathogens cause impetigo? |
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Definition
|
|
Term
| What toxins of S. aureus cause Staphylococcal scalded skin syndrome? |
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Definition
| Exfoliative A and B toxins -> results in epidermolysis of the stratum granulosum |
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Term
| What causes Molluscum contagiosum? What is the name of the cytopasmic inclusion? |
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Definition
| Poxvirus; cytoplasmic inclusions: molluscum bodies |
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Term
| What is a frim, pink. UMBILICATED papule in an immunocompromised person? What is the pathogen that causes this? |
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Definition
| Molluscum contagiosum; poxvirus |
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