Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
1623 Cards in this Set
- Front
- Back
This disease is caused by excess iron |
Hemocrhomatosis, excess iron causes free radical damage |
|
Disorder due to autoimmune production of IgG against platelet antigens (e.g., GPIIb/IIIa) |
Idiopathic thrombocytopenic purpura (ITP) autoantibodies against platelets are made in spleen and the platelets are consumed in the spleen. Therefore removal of spleen can treat chronic ITP. |
|
Pathologic formation of platelet microthrombi in small vessles is known as? |
Microangiopathic hemolytic anemia anemia is caused by the microthrombi shearing the RBCs resulting in hemolytic anemia and schistocytes |
|
Microangiopathic hemolytic anemia that is caused by a decrease in ADAMTS13. ADAMTS13 is used to cleave (deactivate) vWf. This leads to microthrombi due to undegraded ADAMTS13. |
Thrombotic thrombocytopenic purpura -most common cause is autoimmunity to ADAMTS13 |
|
Microangiopathic hemolytic anemia that is caused by endothelial damage by drugs or infection. |
Hemolytic Uremic Syndrome -caused by EColi O157:H7 verotoxin damaging endothelial cells resulting in platelet microthrombi |
|
Syndrome where there is GP1b is deficient leading to platelet adhesion impairment |
Bernard-Soulier syndrome -GP1b on platelets are responsible for binding of vWf
|
|
Syndrome where GP IIb/IIIa is deficient leading to deficient platelet aggregation |
Glanzmann thombasthenia -platelet aggregation is impaired since GPIIb/IIIa is responsible for that |
|
Platelet destruction that arises secondary to heparin therapy is called? |
Heparin-induced thrombocytopenia (HIT) -fragments of destroyed platelets may activate remaining platelets, leading to thrombosis. This is a feared complication. |
|
Pathologic activation of coagulation cascade is called? |
Disseminated Intravascular Coagulation -activation of cascade burns up all the factors and causes microthrombi everywhere as well as bleeding from mucosal surfaces. |
|
This disorder resembles DIC but it has no D-dimers and normal platelet counts |
Disorders of fibrinolysis due to overactive plasmin |
|
What is Virchow's triad? |
The three major risk factors for thrombosis: -Disruption of blood flow -Endothelial cell damage -Hypercoaguable state |
|
What is the mechanism for warfarin skin necrosis? |
Factor 2,7,9,10 and protein C and S need epoxide reductase. Warfarin inhibits this but protein C and S have the shorter half-lives so C and S are inhibited first leading to a hypercoagulable state. |
|
What defect is it when factor V lacks the cleavage site for deactivation by protein C and S? |
Factor V Leiden -most common inherited cause of a hypercoagualble state. |
|
Disorder that when heparin is given it does not incrase PTT? |
ATIII deficiency - anti-thrombin 3 is responsible for inhibited thrombin. Heparin works by activating ATIII. Since there is no ATIII to activate then PTT will remain the same. |
|
Chronic form of gas emobli characterized by multifocal ischemic necrosis of bone. |
Caisson Disease |
|
What are the 4 types of microcytic anemia? |
Iron deficiency Anemia of Chronic Disease Sideroblastic anemia Thallesemia |
|
Increased hepcidin is mechanism for what disease? |
Anemia of chronic disease. Chronic disease state produces hepcidin which sequesters iron into ferritin. So lab values: Ferritin: High TIBC: low Serum iron: low %saturation: low |
|
Iron deficiency anemia has what lab values? |
Ferritin: low TIBC: high Serum iron: low %saturation low |
|
Anemia due to defect in protoporphyrin synthesis is called? |
Sideroblastic anemia |
|
What are lab values for sideroblastic anemia? |
Ferritin: high (macrophages eat all the extra iron) TIBC: low Serum Iron: high %saturation: high
|
|
Anemia due to defect in globin chains. Due to decreased production. |
Thalassemia |
|
Which anemia has target cells? |
B-thallesemia |
|
What are the two causes for macrocytic anemia? |
Folate deficiency Vit B12 deficiency |
|
Two etiologies for normocytic anemia? and how to distinguish? |
peripheral destruction or underproduction. Reticulocyte count. Corrected reticulocyte count >3% suggests peripheral destruction Corrected reticulocyte count <3% suggests underproduction
|
|
Inherited defect of RBC cytoskeleton-membrane tethering proteins leading to normocytic anemia |
Hereditary spherocytosis |
|
Which disease has Howell-Jolly bodies? |
Hereditary spherocytosis Howell-Jowell bodies are DNA in RBCs that are not removed by the spleen. This can occur when the spleen is removed to treat heriditary spherocytosis. |
|
Acquired defect in myeloid stem cells resulting in absent GPI (glycosylphophatidylinositol); renders cells susceptible to destruction by complement. |
Paroxysmal Nocturnal Hemoglobinuria (PNH) -GPI binds DAF and MIRL, these molecules protect it from complement; at night when you breath shallow it causes a slight acidosis which activates complement, w/o DAF and MIRL your cells are attacked. |
|
Inability of Glutathione (an antioxidant) to be regenerated due to a deficiency in an enzyme leading to oxidative stress. |
G6PD deficiency- X-linked recessive disorder resulting in reduced half-life of G6PD; renders cells susceptible to oxidative stress. Glutathione takes the hit from oxidative stress and gets reduced back to glutathione by NADPH; NADPH is produced by G6PD |
|
Which disease has bite cells and Heinz bodies? |
G6PD defiency - Heinz bodies are precipitated Hb due to oxidative stress, when macrophages see those Heinz bodies they bite that part off |
|
Antibody-mediated (IgG or IgM) destruction of RBCs is called? |
Immune hemolytic anemia |
|
Coombs test is used to diagnose what disease? |
Immune hemolytic anemia -direct tests whether the RBCs are bound by IgG -indirect tests whether there are antibodies in the serum that can bind RBCs |
|
What virus infects progenitor RBCs and temporarily halts erythropoiesis? |
Parvovirus B19 |
|
Pathologic process (e.g., metaplastic cancer) that replaces bone marrow; hematopoiesis is impaired, resulting in pancytopenia |
Myelophthisic process |
|
Eosinophilia is classically seen in what disease? |
Hodgkin lymphoma |
|
Basophilia is classically seen in what disease? |
Chronic myeloid leukemia (CML) |
|
Monocytosis is classically seen when? |
Chronic inflammatory states and malignancy |
|
Viruses normally cause lymphocytic leukocytosis, what is one bacteria that can also cause it? |
Bordetella pertussis - this bacteria creates lymphocytosis-promoting factor which blocks circulating lymphocytes from leaving the bleed to enter the lymph node. |
|
EBV infection that results in lymphocytic leukocytosis comprised of reactive CD8+ T cells; CMV is a less common cause. |
Infectious Mononucleosis |
|
How do you differentiate AML vs ALL? |
AML has Myeloperoxidase ALL has TdT+ (DNA polymerase) |
|
Auer rods are made of what, and what do they indicate? |
Auer rods are made of myeloperoxidase and indicate AML. |
|
ALL is associated with what syndrome? |
Down syndrome after the age of 5 |
|
ALL is subdivided into what two categories and how do you distinguish them? |
B-ALL and T-ALL B-All expresses CD10, CD19, and CD20 T-ALL expressed CD2-CD8 |
|
ALL seen in kids has what translocation? |
t(12;21) - good prognosis |
|
ALL seen in adults has what translocation? |
T(9;22) - Philidelphia+ ALL |
|
3T's of acute lymphoblastic lymphoma |
T-ALL Thymic Mass Teenagers |
|
AML more common in children or adults? |
Adults 50-60 years old |
|
Acute promyelocytic leukemia is characterized by what translocation? |
t(15;17) - disrupts retinoic acid receptor, cure is ATRA which is vitamin A, promyelocytes contain Auer rods which increase risk for DIC |
|
Acute monocytic leukemia is a proliferation of what cell and what tissue does it commonly involve? |
Proliferation of monocytes and commonly involves gums |
|
Acute megakaryoblastic leukemia is proliferation of what cell and is associated with what? |
Proliferation of megakaryoblasts and association with down syndrome before age of 5 |
|
Which leukemia is associated with down syndrome before the age of 5 and after the age of 5? |
Acute megakaryoblastic leukemia before age of 5 ALL after age of 5 |
|
What cell is the neoplastic proliferation of CLL? what surface markers are expressed? |
proliferation of naive B-cells, CD5 and CD20 CD5 is normally on T-cells |
|
Smudge cells are classically seen in what disease? |
CLL |
|
Neoplastic proliferation of mature B cells is what disease? |
Hairy Cell Leukemia - characterized by hair cytoplasmic processes |
|
Positive for tartrate-resistant acid phosphatase (TRAP) is indicative of what disease? |
Hairy Cell Leukemia |
|
TRAP mnemonic is for what disease and what are the 3 things it's used for? |
TRAP = Hairy Cell Leukemia Trapped in red pulp (splenomegaly of red pulp when usually its white pulp for leukemias) Trapped in bone marrow - dry tap with bone marrow aspiration Since they are TRAPPED, lymphadenopathy is usually absent |
|
Neoplastic prliferation of mature CD4+ T cells is called? |
ATLL - Adult T-cell Leukemia/Lymphoma |
|
ATLL is associated with what virus? |
HTLV-1 - most commonly seen in Japan, Human lymphotropic virus 1 |
|
Pt with lytic bone lesions with hypercalcemia WITH a RASH |
ATLL (Adult t-cell leukemia/lymphoma) - if without a rash then think multiple myeloma |
|
Neoplastic proliferation of mature CD4+ T cells that infiltrate the skin |
Mycosis fungoides |
|
What are aggregates of neoplastic cells in the epidermis called? |
Pautrier microabscesses |
|
Neoplastic proliferation of mature myeloid cells, especially granulocytes, basophils are characteristically increased. |
CML - Chronic Myeloid Leukemia |
|
CML is driven by what translocation? |
t(9;22), philidelphia chromosome |
|
Neoplastic proliferation of myeloid cells, especially RBCs |
Polycythemia Vera |
|
What are the three different causes for painless lymphadenopathy |
Chronic inflammation metastatic carcinoma lymphoma |
|
Patients with rheumatoid arthritis have expansion in what region of the lymph node? |
Follicle |
|
Patients with early HIV have expansion in what region of the lymph node? |
Follicle |
|
Patients with a viral infection have expansion in what region of the lymph node? |
Paracortex - where the T-cells live |
|
Follicular lymphoma is driven by what translocation? and what genes are traded? |
t(14;18) -14 has Ig heavy chain, 18 is Bcl-2, Bcl-2 is used to stabilize the mitochondrial membrane which prevents cytochrome C from leaking which prevents apoptosis. |
|
Most feared complication of follicular lymphoma? |
Having it progress to diffuse large B-cell lymphoma |
|
Mantle cell lymphoma, marginal zone lymphoma, and follicular have expansion of what cell type? |
Neoplastic small B cells (CD20+) |
|
Mantle Cell lymphoma is driven by what translocation? and what genes are being switched? |
t(11;14) - 11 Cyclin D1, 14 has Ig heavy chain. Cyclin D1 promotes G1/S transition in cell cycle |
|
Neoplastic INTERMEDIATE-sized B Cells (CD20+T that is associated with EBV? |
Burkitt Lymphoma |
|
African form of Burkitt lymphoma most commonly arises where? |
In the jaw |
|
Sporadic form of Burkitt lymphoma most commonly arises where? |
abdomen |
|
Burkitt Lymphoma is driven by what translocation? |
t(8;14) - 8 is c-myc and 14 is Ig heavy chain c-myc oncogene promotes cell growth |
|
What disease has a starry sky appearance? |
Burkitt Lymphoma |
|
Neoplastic large B-cell (CD20+) that grow diffusely in sheets. |
Diffuse Large B-cell Lymphoma |
|
Most common form of non-hodgkin lymphoma? |
Diffuse Large B-cell lymphoma |
|
Neoplastic proliferation of Reed-Sternberg cells |
Hodgkin Lymphoma |
|
Reed-Sternberg cells have what surface markers? |
CD15 and CD30 |
|
Which disease has owl-eye nulclei? |
Reed-sternberg cells of hodgkin lymphoma |
|
Which interleukin is high in multiple myeloma |
IL-6 |
|
What disease has an increased serum protein with M spike on SPEP(serum protein electrophoresis) but it has none of the other features of multiple myeloma (no lytic lesions, hypercalcemia, AL amyloid, or Bence-Jones proteinuria) |
Monoconal Gammopathy of Undetermined Significance |
|
Waldenstrom Macroglobulinemia has what type of lymphoma with what type of Ig production? |
B-cell lymphoma with monoclonal IgM production |
|
Neoplastic proliferation of Langerhans Cell |
Langerhans Cell histiocytosis |
|
Birbeck granules (tennis racquet) is characteristic of what disease? |
Langerhans Cell histiocytosis |
|
2 malignant disease of langerhans cells? |
Letterer-Siwe disease Hand-Schuller-Christian disease |
|
Benign proliferation of langerhans cells in bone |
Eosinophlic granuloma |
|
WBC differential from highest to lowest? |
Mnemonic: Neutrophils Like Making Everything Better Neutrophils (54-62%) Lymphocytes (25-33) Monocytes (3-7) Eosinophils (1-3) Basophils (0-.75)
|
|
CD14 is cell surface marker for which cell? |
Macrophages |
|
Target Cell is associated with what pathology? (4) |
"HALT" said the hunter to his target HbC disease Asplenia Liver disease (alcoholism) Thalassemia |
|
What pathology has glutamic acid to lysin mutation at residue 6 in Beta globin? |
HbC defect - extravascular normocytic anemia |
|
Apixaban and rivaroxaban Drug class and clinical use |
Class: Direct Factor Xa inhibitor Mechanism:Bind and directly inhibit the activity of factor Xa C:DVT and PE |
|
Clopidogrel Drug class and clinical use |
Drug class: ADP receptor inhibitor
M: inhibit platelet aggregation by irreversibly blocking ADP receptors C: acute coronary syndrome, coronary stent, prophy for recurrent strokes T: TTP/HUS may be seen |
|
Ticlopidine Drug class and clinical use |
Drug class: ADP receptor inhibitor
M: inhibit platelet aggregation by irreversibly blocking ADP receptors C: acute coronary syndrome, coronary stent, prophy for recurrent strokes T: TTP/HUS may be seen, NEUTROPENIA |
|
Abciximab Drug class and clinical use |
GP IIb/IIIa inhibitors M: prevent aggregation of platelets Clinical Use: unstable angina, precutaneous transluminal coronary angioplasty |
|
Eptifibatide Drug class and clinical use |
GP IIb/IIIa inhibitors M: prevent aggregation of platelets Clinical Use: unstable angina, precutaneous transluminal coronary angioplasty |
|
Which cells are infected in infectious mononucelosis? |
CD8+ T-cells |
|
Hypersegmented neutrophils and elevated MCV is associated with what? |
Folate or B12 deficient anemias |
|
Which blood thinner has increased risk for neutropenia? |
Ticlopidine - ADP receptor inhibitor which inhibits platelet aggregation |
|
What is the mechanism for hydroxyurea in the tx of sickle cell anemia? |
It increases the formation of HbF which do not sickle. |
|
What drug is used for Heparin induced thrombocytopenia? and what is it's mechanism of action? |
Argatroban which is a direct thrombin inhibitor. Bivalriduin can also be used. |
|
Pt with hemolytic anemia, hypercoaguable state, and thrombocytopenia suggests what disease? |
Paroxysmal Hemolytic Anemia |
|
Which anemia has increased MCHC (mean corpuscular hemoglobin concentration)? |
Hereditary Spherocytosis |
|
Which anemia has osmotic fragility? |
Hereditary Spherocytosis |
|
What drug reverses heparin? |
Protamine |
|
What causes a left shift in oxygen-hemoglobin dissociation curve? Right shift? |
Left shift = lower Base Lower 2,3 DPG Lower Temperature Right shift is opposite Acid Higher 2,3 DPG Higher temperature
CADET face RIGHT CO2 Acid DPG Exercise Temperature |
|
What are the acute-phase reactants of inflammation? (4) |
IL-6 IL-1 TNF-a IFN-y |
|
Granulomatous vasculitis that classically involves branches of the carotid artery |
Temporal (Giant Cell) Arteritis |
|
Granulomatous vasculitis that classically involves the aortic arch at branch points. |
Takayasu Arteritis |
|
Tx for granulomatous vasculitis such as Temporal (giant cell) arteritis or Takayasu arteritis |
Corticosteroids |
|
Pts with Temporal Giant Cell Arteritis have a high risk for what complication? |
Blindness |
|
Medium-Vessel Vasculitis involves what portion of the arteries? |
It commonly involves the muscular arteries that supply the organs |
|
(Medium-Vessel) Necrotizing vasculitis involving multiple organs; lungs are spared. |
Polyarteritis Nodosa |
|
This medium vessel vasculitis is associted with serum HBsAG (Hepatitis B antigen) |
Polyarteritis Nodosa |
|
This disease has a string-of-pearls appearance on imaging |
Polyarteritis Nodosa |
|
Medium-Vessel vasculitis that affects asian children <4 years old |
Kawasaki Disease |
|
Medium-Vessel vasculitis that commonly involves the coronary artery |
Kawasaki disease |
|
Medium-Vessel vasculitis that is highly associated with heavy smoking |
Buerger Disease |
|
What is the treatment for kawasaki disease? |
Aspirin |
|
Rash on the palms and rash on the soles is associated with what medium-vessel vasculitis? |
Kawasaki disease |
|
Necrotizing vasculitis that involves the digits |
Buerger Disease |
|
Necrotizing granulomatous vasculitis the involves the nasopharynx, lungs, and kidneys |
Wegener Granulomatosis |
|
Wegener granulomatosis involves what parts of the body? |
Nasopharynx, lungs, and kidneys |
|
c-ANCA is associated with which small-vessel vasculitis? |
Wegener Granulomatosis |
|
Necrotizing vasculitis involving multiple organs, especially lung and kidney. |
Microscopic Polyangitis |
|
Serum p-ANCA is associated with which small-vessel vasculitis (2)? |
Microscopic polyangitis Churg-Strauss syndrome |
|
Necrotizing granulomatous vasculitis with eosinophils |
Churg-Strauss syndrome |
|
Which small-vessel vasculitis have granulomas? |
Wegener and Churg-Strauss |
|
Vasculitis due to IgA immune complex depositiion; most common vasculitis in children |
Henoch-Schnolein purpura |
|
This disease presents with palpable purpura on buttocks and legs, GI pain and bleeding. |
Henoch-Schnolein purpura |
|
This small0-vessel vasculitis presents with hematuria (IgA nephropathy) |
Henoch-Schnolein purpura |
|
How does angiotensin II increase blood pressure? |
-Contracts arteriolar smooth muscle increasing TPR -Promotes adrenal release of aldosterone, which increases resorption of sodium in the distal convoluted tubule |
|
Two causes for renal artery stenosis, one is for elderly males and one is for young females. |
Elderly male - atherosclerosis Young female - fibromuscular dysplasia |
|
Which portion of the artery becomes thickened in atherosclerosis? |
Intima |
|
Hyaline arterololsclerosis can be caused by what two things? and what does this lead to? |
Benign HTN - increased pressure forces proteins into the vessel wall Diabetes - non-enzymatic glycosylation making membrane leaky
Leads to arteriolonephrosclerosis --> chronic renal failure |
|
Hyperplastic arteriosclerosis is caused by what? and what does it lead to? |
Malignant HTN Acute renal failure |
|
Calcification of the media of muscular arteries; non-obstructive; not clinically significant |
Monckeberg Medial Calcific Sclerosis |
|
Intimal tear with dissection of blood through media of aortic wall is called? |
Aortic Dissection |
|
Most common cause of Aortic Dissection |
HTN |
|
Balloon-like dilation of the thoracic aorta is called |
Thoracic aneurysm |
|
Balloon-like dilation of the abdominal aorta is called? |
Abdominal aneurysm |
|
Classic cause of thoracic aneurysm is what? |
Tertiary syphillis - endarteritis of the vaso vasorum results in luminal narrowing and atrophy of the vessel wall |
|
"Tree bark" appearance of aorta is seen in what? |
Thoracic aneurysm due to syphillis |
|
Where do abdominal aortic aneurysms usually occur? |
below renal arteries but above aortic bifurcation |
|
Abddominal aortic aneurysm usually caused by what? |
HTN Classically seen in male smokers > 60 years old with HTN |
|
Benign tumor comprised of blood vessels? |
Hemangioma |
|
Pt present with hypotension, pulsatile abdominal mass, and flank pain. What is the dx? |
Abdominal aortic aneurysm rupture |
|
Malingnant proliferation of endothelial cells |
Angiosarcoma |
|
This cancer is associated with exposure to PVC |
Liver angiosarcoma |
|
This cancer is associated with arsenic and Thorotrast |
Liver angiosarcoma |
|
Low-grade malignant proliferation of endothelial cells associated with HHV-8 |
Kaposi Sarcoma |
|
What percentage of blood flow must be stopped before symptoms emerge? |
70% stenosis; stable angina does not have symptoms unless there is 70% blockage |
|
Which angina has the EKG show ST-segment depression and which has EKG elevation? (3) |
Stable and unstable have depression due to subendocardial ischemia, prinzmetal has elevation due to transural ischemia |
|
Myocardial Infarction usually affects what portion of the heart? |
Left Ventricle |
|
Most sensitive and specific marker for a myocardial infarction |
Troponin I |
|
What marker is useful for detecting reinfarction days after MI? |
CK-MB, it returns to normal by 72 hours while Troponin I stays high for 7-10 days. |
|
Exposure of pericardium to immune system leading to autoimmunity leading to pericarditis |
Dressler syndrome |
|
Stages of MI, 1 day, 1 week, 1 month |
Coag Necrosis | Neutro then Macro | Granulation Tissue | Scar Tissue |
|
What is the main tx for Left-Sided Heart Failure? |
ACE inhibitor |
|
What is the most common cause for Right-sided heart failure? |
Left-side Heart failure |
|
Nutmeg liver is associated with what? |
Right-sided heart failure |
|
Defect in the septum that divides the right and left ventricles |
Ventricular Septal Defect |
|
What is Ventricular septal defect associated with? |
Fetal alcohol syndrome |
|
5 T's of Right to left shunt |
Truncus arteriosus Transposition of great vessels Tricuspid atresia Tetralogy of fallot Total anomalous pulmonary venous return |
|
Two types of Atrial septal defect: which is most common, which is associated with Down syndrome? |
Ostium secundum = most common Ostium primium = Down syndrome |
|
Patent ductus arteriosus is associated with what? |
Rubella |
|
What is Eisenmenger syndrome? |
When a Left to right shunt reverse to a right to left shunt, leading to cyanosis. |
|
What is the tetrology of Fallot? |
PROV Pulmonary stenosis Right Ventricular Hypertrophy Overriding AORTA Ventricular Septal defect |
|
Which congenital defect which becomes cyanotic stops the cyanosis when they squat? |
Tetralogy of Fallot |
|
Boot shaped heart on X-ray is associated with what? |
Tetralogy of Fallot |
|
Transposition of the great vessels is associated with what? |
Maternal diabetes |
|
Infantile form of Coarction of Aorta is associated with what two things and is located where on the aortic arch? |
Infantile associated with PDA and Turner syndrome and lies distal to the aortic arch but proximal to the PDA |
|
Adult form of coarction of aorta is located where and associated with what? |
Distal to the aortic arch, there is no PDA. Associated with bicuspid aortic valve |
|
This present with HTN in upper extremities and hypotension with weak pulse in lower extremities |
Adult coarction of aorta. This is because the blockage is after the aortic arch. Upper extremities gets all the blood pressure while the lower extremities are blocked by the coarction. |
|
Collateral circulation develops across intercostal arteries, engorged arteries causes nothing of ribs on x-ray. What pathology is this? |
Adult coarctation of aorta |
|
Systemic complication of pharyngitis due to group A B-hemolytic streptococci |
Acute rheumatic fever |
|
What is the pathophysiology of acute rheumatic fever? |
Caused by molecular mimicry; bacterial M protein resembles human tissues |
|
What is Jones criteria used for? |
Acute Rheumatic Fever |
|
What are the Major criteria for Acute Rheumatic Fever? |
Joints - migratory polyarthritis O = heart problems - pancarditis Nodules = Subcutaneous nodules E = Erythema Marginatum Sydenham chorea = rapid, involuntary muscle movement |
|
In acute rheumatic fever which valve is most often involved? and if another valve is involved which one? |
Alway Mitral valve maybe also aortic valve |
|
Acute Rheumatic fever has these two histological characteristics |
Aschoff body (foci of chronic inflammation) involving myocardium with anitschkow cells (caterpillar nuclei) |
|
Valve scarring that arises as a consequence of rheumatic fever |
Chronic Rheumatic Heart Disease |
|
Narrowing of the aortic valve orifice |
Aortic Stenosis |
|
Aortic stenosis is caused by what? |
Fibrosis and calcification from "wear and tear" |
|
Backflow of blood from aorta into the left ventricle during diastole |
Aortic Regurgitation |
|
Two causes for aortic regurgitation |
Aortic root dilation (e.g., syphilitic aneurysm) or valve damage (e.g., infective endocarditis) |
|
Hyperdynamic circulation is associated with what? |
Aortic Regurgitation |
|
Ballooning of mitral valve into left atrium during systole |
Mitral Valve Prolapse |
|
Myxoid degneration of mitral valve will lead to what? |
Mitral valve prolapse |
|
Reflux of blood from left ventricle into the left atrium during sytole |
Mitral regurgitation |
|
Narrowing of the mitral valve orifice |
Mitral Stenosis |
|
Mitral stenosis is usually due to? |
Chronic rheumatic valve disease Acute rheumatic heart disease leads to mitral regurgitation. |
|
Most common overall cause of endocardittis |
Streptococcus viridans |
|
Most common cause and valve affected in endocarditis in IV drug users? |
S. Aureus, Tricuspid valve |
|
This bacteria is associated with prosthetic valves |
S. Epidermidis |
|
Endocarditis in patients due to S. Bovis usually have what other pathology? |
Colorectal carcinoma |
|
What are the HACEK organisms and what is the signficance? |
Haemophilus Actinobacillus Cardiobacterium Eikenella Kingella
This organisms are associated with endocarditis with negative blood cultures |
|
Janeway lesions (erythematous non-tender lesions on the palms and soles) is a clinical feature of what? |
Bacterial Endocarditis |
|
Osler nodes (tender lesions on fingers and toes) is a clinical feature of what? |
Bacterial Endocarditis |
|
Sterile vegatations that arise with hypercoaguable state or underlying adenocarcinoma |
Nonbacterial thrombotic endocarditis |
|
Sterile vegetations associated with SLE |
Libman-Sacks Endocarditis |
|
Dilation of all four chambers of heart |
Dilated cardiomyopathy |
|
Massive hypertrophy of the left ventricle usually due to genetic mutations in sarcomere proteins; most common form is autosomal dominant |
Hypertrophic cardiomyopathy |
|
Sudden death due to ventricular arrhythmias; common cause of sudden death in young adults |
Hypertrophic cardiomyopathy |
|
Decreased compliance of the ventricular endomyocardium that restricts filling during diastole |
Restrictive Cardiomyopathy |
|
Benign mesenchymal tumor with a gelatinous appearance and abundant ground substance on histology |
Myxoma |
|
Restrictive cardiomyopathy presents as what? |
Congestive heart failure |
|
Most common primary cardiac tumor in adults |
Myxoma |
|
Usually forms a pedunculated mass in the left atrium that causes syncope due to obstruction of mitral valve |
Myxoma |
|
This cardiac tumor is associated with tuberous sclerosis |
Rhabdomyoma |
|
Benign hamartoma of cardiac muscle |
Rhabdomyoma |
|
Fetal erythropoiesis order of organs responsible. (4) |
Yolk Sac (3-8wks) Liver (6wks-birth) Spleen (10-28wks) Bone Marrow (18wks to adult) Young Liver Synthesizes Blood |
|
What are the components of fetal hemoglobin and adult hemoglobin? |
A2Y2 (alpha2 gamma2) = fetal A2B2 (alpha2 beta2) = adult |
|
Venodilators decrease preload or afterload? |
Venodilators decrease preload |
|
Vasodilators decrease preload or afterload? |
Vasodilators decrease afterload |
|
S1 and S2 sound is due to and where is it located? |
S1 - Mitral and tricuspid valve closure, loudest at mitral area S2 - Aortic and pulmonary valve closure. Loudest at left sternal border |
|
S3 and S4 sound due to? |
S3 - in early diastole during rapid ventricular filling phase. S4 - "atrial kick" in late diastole. High atrial pressure. Associated with ventricular hypertrophy. |
|
Receptors in aortic arch transmit via what nerve? |
Vagus (responds only to increase in BP) |
|
Receptors in carotid sinus transmit via what nerve? |
Glossopharyngeal (responds to decrease and increase of BP) |
|
HTN with CHF, cautious of what drug? |
B-Blockers |
|
HTN with Diabetes, should use what drug? |
ACE inhibitors/ARBs are protective against diabetic nephropathy |
|
HMG-CoA reductase inhibitors (statins) effect on LDL, HDL, Triglycerides, MOA, side effects |
3x decrease of LDL, 1x increase of HDL, 1x decrease of triglycerides. Inhibit HMG-COA reductase, hepatoxicity, rhabdomyolysis |
|
Niacin (Vit B3) effect on LDL, HDL, Triglyceride, MOA, side effects |
2x decrease LDL, 2x increase HDL, 1x decrease triglyceride. Inhibit lipolysis in adipose tissue, reduce hepatic VLDL synthesis. Red, flushed face which is decreased by aspirin. |
|
Bile acid resins (cholestyramine, colestipol, colesevelam) effect on LDL, HDL, Triglyceride, MOA, side effect |
2x decrease LDL, slight increase HDL, slight INCREASE of triglyceride. Prevent intestinal reabsorption of bile acids; liver must use cholesterol to make more. Decrease absorption of fat-soluble vitamins. Gall stones. |
|
Cholesterol absorption blockers (ezetimibe) effect on LDL, HDL, Tri, MOA, side effect |
2x decrease LDL, no effect on HDL or Tri, Prevent cholesterol absorption at small intestine brush border. |
|
Fibrates (gemfibrozil, clofibrate, -fibrates) effect on LDL, HDL, TRI, MOA, side effect. |
decrease LDL, increase HDL, 3x decrease Tri. Upregulate LPL--> TG clearance activates PPAR-a to induce HDL synthesis |
|
Which Lipid-lowering agent class best decreases Triglycerides? |
Fibrates (Gemfibrozil, clofibrate, bezafibrate, fenofibrate) |
|
Which lipid-lowering agent class best decreases LDL? |
HMG-CoA reductase (statins) |
|
Which lipid-lowering agent increases triglycerides? |
Bile acid resins (Cholestyramine, colestipol, colesevelam) |
|
Which lipid lower agent has no effect on HDL or Triglycerides? |
Cholesterol absorption blockers (ezetimibe) |
|
Statins in combination with what drugs has increased risk for rhabdomyolysis? |
Statins with fibrates and niacin |
|
When determining metabolic, respiratory, alka vs acidosis what are the steps? |
Look at pH to determine if its alka or acid, now determine if its resp or meta by looking at bicarbonate. |
|
How do beta blockers effect the RAAS? |
Beta blockers inhibit renin release by blocking beta-1 receptors on Juxtaglomerular cells |
|
Most common benign vascular tumor in children? adults? |
Strawberry hemangioma - children Cherry hemangioma - adults |
|
Anti-hypertensives ending in -zosin have what MOA? |
They are alpha-1 blockers and are also useful for benign prostatic hyperplasia |
|
If you have a child with nasal polyps what should you also be checking for? |
Cystic fibrosis |
|
Pts with ASA-intolerant asthma have what triad? |
Asthma, aspirin-induced bronchospasms, and nasal polyps |
|
Benign tumor of nasal mucosa composed of large blood vessels and fibrous tisse |
Angiofibroma |
|
If an adolescent male has profuse epitaxis what should you suspect? |
Angiofibroma |
|
Malignant tumor of nasopharyngeal epithelium |
Nasopharyngeal carcinoma |
|
Nasopharyngeal carcinoma is associated with what? |
EBV |
|
Nasopharyngeal carcinoma has what type of histology? |
Pleomorphic KERATIN-POSITIVE epithelial cells |
|
Inflammation of the upper airway |
Laryngotracheobronchitis (Croup) |
|
Nodule that arises on the true vocal cord |
Vocal cord nodule |
|
Benign papillary tumor of the vocal cord |
Laryngeal papilloma |
|
Laryngeal papilloma is due to what? |
HPV 6 and 11 |
|
Laryngeal papillomas are usually (single/multiple) in adults and (single/multiple) in children |
Single in adults Multiple in children |
|
Squamous cell carcinoma arising from epithelial lining of vocal cord |
Laryngeal carcinoma |
|
2 key chemical mediators for pain |
Prostaglandin E2 and Bradykinin |
|
Three patterns of pneumonia that can be seen on chest x-ray |
Lobar pneumonia, bronchopneumonia, interstitial pneumonia |
|
Pneumonia characterized by consolidation of an entire lobe of the lung |
Lobar pneumonia |
|
Pneumonia characterized by scattered patchy consolidation centered around bronchioles; often multifocal and bilateral |
Bronchopneumonia |
|
Pneumonia characterized by diffuse interstitial infiltrates. |
Interstitial (atypical) pneumonia |
|
Lobar and bronchopnemonia usually bacterial or viral? |
Bacterial |
|
Interstitial pneumonia is usually bacterial or viral? |
viral |
|
Most common cause for lobar pneumonia (2 bacterias)? |
Streptococcus Pneumoniae (95%) and Klebsiella pneumoniae |
|
Lobar pneumonia in patients who have high chance of enteric flora aspiration (elderly in nursing homes, alcoholics). What is the bacterial cause of the pneumonia? |
Klebsiella pneumoniae |
|
Which type of pneumocyte can replicate Type 1 or 2? |
Type 2 can replicate |
|
Which type of pneumocyte is responsible for producing surfactant? |
Type 2 |
|
Which type of pneumocyte is responsible for the epithelial lining? |
Type 1 |
|
What are the four classic phases of lobar pneumonia and describe each. |
Congestion - congested vessels and edma Red hepatization - exudate, neutrophils, and hemmorrhage filling the alveolar air space Gray hepatization - degradation of red cells within exudate Resolution - regeneration |
|
Aspiration pneumonia usually affects which side? |
Right lower lobe (due to anatomy) |
|
Which bacteria anaerobic bacteria are normally responsible for aspiration pneumonia? (3) |
Bacteroidies, fusobacterium, and peptococcus |
|
Most common site for systemic spread of TB? |
Kidneys creating a sterile pyuria |
|
Which congenital heart defects are caused by abnormal migration of neural crest cells (3)? |
Truncus Arteriosus Transposition of Great Vessels Tetralogy of Fallot
|
|
What is the DOC for Beta blocker overdose? and what is the receptor it works on? |
Glucagon, which increases HR and contractility via cAMP. |
|
T-lymphocyte immunodeficiency that resultsin maldevelopment of the third and fourth branchial (pharyngeal) pouches. |
DiGeorge syndrome |
|
DiGeorge syndrome is due to a deletion of what chromosome? |
Chromsome 22 |
|
MOA of Dobutamine |
B1-adrenergic receptor agonist. Is used for acute heart failure due to decreased myocardial contractility |
|
Beta-Blockers work where on an EKG? |
PR interval, they slow AV conduction. |
|
(4)Hyper and (2)Hypo problems of Thiazide diuretics |
Hyperuricemia Hypercalcemia Hyperglycemia Hyperlipidemia
Hypokalemia Hypotension |
|
Doxorubicin can causes what problem with the heart? |
Dilated cardiomyopathy |
|
Sharp pain that is relieved by sitting up and leaning forward suggests what? |
Pericarditis - associated with SLE |
|
Nitrates mixed with drugs for erectile dysfunction are absolutely contraindicated, what is the MOA? |
Increased cyclic GMP due to nitrates synthesizing and PDE inhibitors inhibiting degradation. |
|
What is FVC? Normal amount? |
Forced vital capacity - breathe in all the way breath out all the way. 5L |
|
What is FEV1? Normal amount? |
Forced Expiratory Volume - How much air you can breath out in the first second. 4L |
|
In airway obstruction what are the relative decreases in FVC, FEV1, and FEV1:FVC ratio,FRC, TLC? |
FVC - decreases FEV1 - significant decrease FEV1:FVC ratio - decreases FRC - Increased TLC - increases due to trapped air |
|
Chronic productive cough lasting at least 3 months over a minimum of 2 years; highly associated with smoking |
Chronic bronchitis |
|
Destruction of alveolar air sacs leading to COPD |
Emphysema |
|
What enzyme neutralizes proteases in alveolar air sacs? |
A1AT - a1-antitrypsin neutralizes proteases |
|
Deficiency in what can cause emphysema? |
A1AT - a1-antitrypsin |
|
Barrel-chest (increased AP diameter) is found in emphysema or chronic bronchitis? |
Emphysema |
|
Smoking leads to centriacinar or panacinar emphysema? |
centriacinar |
|
A1AT deficiency leads to centriacinar or panacinar emphysema? |
panacinar |
|
In A1AT, the two organs affected are what? |
Lung emphysema and liver cirrhosis |
|
Biopsy of liver in A1AT will reveal what? |
pink, PAS-positive globules in hepatocytes |
|
Prolonged expiration with pursed lips, is which COPD? |
Emphysema |
|
Match: Emphysema and Cystic fibrosis with Increased or Decreased Functional Residual Capacity |
Emphysema = Increased FRC Cystic Fibrosis = Decreased FRC |
|
Key cytokines released by Th2 helper CD4+ T cells? |
IL-4, IL-5, IL-10 |
|
IL-5 main job? |
recruit eosinophils |
|
In asthma allergens stimulate what cells to release what three cytokines which are responsible for what? |
Th2 CD4+ T cells --> IL-4,IL-5,IL-10 IL-4 = IgE Class switch IL-5 = recruit eosinophils IL-10 = inhibit Th1 stimulate Th2 |
|
Reexposure to allergen has two phases, what is released in the first phase, what is released in the second phase? |
First phase = release of histamine Second phase = Release of leukotriene C4,D4,E4 |
|
What are leukotrienes C4,D4,E4 responsible for? |
Bronchoconstriction, inflammation, edema |
|
What are Charcot-Ledyden crystals and what are they assocaited with? |
Eosinophil-derived crystals associated with asthma |
|
What are Curschmann spirals? |
spiral-shaped mucus plugs found in asthma |
|
What is permanent dilation of bronchioles and bronchi? |
Bronchiectasis |
|
What is the classic cause of bronchiectasis? |
Classic cause is cystic fibrosis |
|
What syndrome is associated with Bronchiectasis, and what is defective? |
Kartagener syndrome - defect in dynein arm of cilia |
|
In restricted lung diseases are these values increased or decreased? TLC, FVC, FEV1,FRC, FEV1:FVC ratio |
TLC - decreased FVC - decreased 2x FEV1 - decreased FRC - decreased FEV:FVC ratio - increased |
|
Fibrosis of lung interstitium with unknown etiology |
Idiopathic Pulmonary Fibrosis |
|
What chemical mediator drives the process of idiopathic pulmonary fibrosis? |
TGF-B |
|
What is interstitial fibrosis due to occupational exposure |
Pneumoconioses |
|
What are the 4 types of Pneumoconioses? |
Coal Worker's Silicosis - sandblasters and silica miners Beryliosis - berylium miners and aerospace Absestosis - construction workers, plumbers, shipyard workers |
|
Aerospace worker who has lung problmes, what is the dx? |
Berylliosis |
|
What is the name of collections of carbon-laden macrophages, like that seen in Coal Worker's pneumoconiosis? |
Anthracosis |
|
What pneumoconioses increases risk for TB and why? |
Silicosis - Silica impairs phagolysosome formation by macrophages. |
|
Which pneumoconioses has noncaseating granulomas in the lung, hilar lymph nodes, and systemic organs, similar to sarcoidosis? |
Berylliosis - distinguish by med hx and see if they work in the aerospace industry |
|
Absetosis more likely to get lung carcinoma or mesothelioma? |
Lung carcinoma |
|
Systemic disease characterized by noncaseating granulomas in multiple organs |
Sarcoidosis |
|
Asteroid bodies are associated with what disease? |
Sarcoidosis - stellate inclusions that are often seen within giant cells of the granulomas |
|
Sarcoidosis has two lab findings what are they? |
Elevated serum ACE Hypercalcemia |
|
What is the pathophysiology of hypercalcemia in sarcoidosis? |
1-alpha hydroxylase activity of epitheliod histiocytes converts vitamin D to its active form - this occurs in all non-caseating granulomas (e.g., sarcoidosis and berylliosis) |
|
Granulomatous reaction to inhaled organic antigens where chronic exposure leads to interstitial fibrosis. |
Hypersensitivity Pneumonitis |
|
Aschoff body and anischkow (caterpillar) cells are associated with what? |
Acute Rheumatic Fever |
|
What are plexiform lesions and what are they associated with? |
Tufts of capillaries; associated with pulmonary HTN |
|
Pulmonary HTN leads to what two things? |
Right ventricular hypertrophy and cor pulmonale |
|
If someone presents with exertional dyspnea what should come to mind? |
Pulmonary HTN |
|
Primary pulmonary HTN the etiology is unknown but some familial forms are related to inactivating mutations of what, which leads to what? |
BMPR2, leading to proliferation of vascular smooth muscles |
|
Diffuse damage to the alveolar-capillary interface leads to formation of what? What is this disease called? |
Leads to formation of hylaine membranes in alveoli, disease is Acute Respiratory Distress Syndrome |
|
Diffuse white-out on chest x-ray suggests what? |
Acute Respiratory Distress Syndrome |
|
Which pneumocytes get damaged in Acute Respiratory Distress syndrome? |
Type I and Type II both get damaged |
|
Respiratory distress due to inadequate surfactant levels is called? |
Neonatal Respiratory Distress syndrome |
|
Neonatal Respiratory Distress Syndrome associated with what 3 things? |
Prematurity C-Section Maternal Diabetes |
|
What is the major component of surfactant? |
Phosphatidylcholine (lecithin) |
|
Why does C-section delivery cause increased risk for Neonatal Respiratory Distress Syndrome? |
Lack of stress-induced steroids; steroids increases synthesis of surfactant |
|
What is particularly carcinogenic in cigarette smoke? |
Polycyclic aromatic hydrocarbons |
|
What type of cancer is increased in radon miners? |
Lung cancer |
|
Two benign "coin-lesions" that can be seen on a chest x-ray |
Granuloma and Bronchial Hamartoma |
|
What is a benign tumor composed of lung tissue and cartilage; often calcified on imaging? |
Bronchial Hamartoma |
|
Lung carcinoma is divided into what two categories? and what is the significance? |
Small cell carcinoma and non-small cell carcinoma. Small cell responds to chemo, non-small cell responds to surgery |
|
Non-small cell carcinoma with glands or mucin |
Adenocarcinoma |
|
Non-small cell carcinoma with keratin pearls or intracellular bridges |
Squamous cell carcinoma |
|
Non-small cell carcinoma with NO keratin pearls, intracellular bridges, glands, or mucin |
Large cell carcinoma |
|
Small cell carcinoma is associated with what syndrome? |
Eaton-Lambert syndrome - anti-bodies against calcium channels in NMJ leading to weakness |
|
Lung carcinomas that start with S (small cell carcinoma, squamous cell carcinoma) have what common attributes? (3) |
Smokers, Central, paraneoplastic syndromes |
|
Which lung cancer can cause hypercalcemia and what is the mechanism? |
Squamous cell carcinoma due to production of PTHrP (parathyroid hormone related Peptide) |
|
Which lung cancer may produce ADH or ACTH? |
Small cell carcinoma |
|
What is the most common tumor in non-smokers? |
Adenocarcinoma |
|
What is the most common tumor in female smokers? |
Adenocarcinoma |
|
What is the most common tumor in male smokers? |
Squamous cell carcinoma |
|
Name the tumor: Columnar cells that grow along preexisting bronchioles and alveoli; arises from Clara cells |
Bronchioalveolar carcinoma |
|
Which lung cancers are chromogranin positive? |
Carcinoid tumor and Small Cell Carcinoma |
|
Lung cancer with well differentiated neuroendocrine cells |
Carcinoid tumor |
|
Two lung cancers not related to smoking |
Bronchioalveolar carcinoma Carcinoid tumor |
|
Two lung cancers that are derived from neuroendocrine cells; what is the difference? |
Small cell carcinoma - poorly differentiated Carcinoid tumor - well differentiated |
|
Which lung cancer presents as a polyp-like mass in the bronchus? |
Carcinoid tumor |
|
Two Most common source for metastasis to lung? |
Breast and colon cancer |
|
This lung cancer has multiple 'cannon-ball' nodules on imaging |
Metastasis to lung |
|
Where do lung cancers like to metastasize? |
Adrenal gland |
|
Lung cancer can lead to Horner syndrome, what are the 3 characteristics? |
Compression of sympathetic chain leads to -ptosis (drooping of eyelid) -miosis (pinpoint pupil) -anhidrosis (no sweating) |
|
Accumulation of air in the pleural space is called? |
Pneumothorax |
|
Pneumothorax caused by rupture of an emphysematous bleb |
Spontaneous pneumothrax |
|
Pneumothorax caused by penetration of chess wall |
Tension pneumothorax |
|
Malignant neoplasm of mesothelial cells of lung |
Mesothelioma |
|
Structures that perforate the diaphragm at T8, T10, T12? |
T8: IVC T10: esophagus, (CNX vagus), T12: aorta, thoracic duct, azygos vein |
|
What nerves innervate diaphragm? |
C3,4,5 |
|
Fetal Hb has higher or lower affinity for 2,3-BPG? what does this mean? |
lower affinity for 2,3-BPG which means it has a higher affinity for O2. |
|
Pancoast tumors can have what 4 complications? |
Horner syndrome SVC syndrome Sensorimotor deficits Hoarseness (recurrent laryngeal nerve) |
|
Ethambutol can be used to treat TB; what is it's most notable complication? |
Optic neuritis |
|
What is S. pneumoniae's main virulence factor? |
Having an outer polysaccharide capsule which prevents phagocytosis |
|
What should you think of when you see "new screening test" |
Lead time bias |
|
Arterial blood gas for Metabolic acidosis (pH, pCO2, HCO3-) |
pH decreased PCO2 decreased HCO3- decreased |
|
Arterial blood gas for Respiratory Acidosis (pH, pCO2, HCO3-) |
pH decreased PCO2 increased HCO3 normal or increased(compensated) |
|
Arterial blood gas for Metabolic alkalosis (pH, pCO2, HCO3-) |
pH increased PCO2 increased HCO3 increased |
|
Arterial blood gas for Respiratory alkalosis (pH, pCO2, HCO3-) |
pH increased PCO2 decreased HCO3 decreased(compensated) or normal |
|
Diabetic ketoacidosis leads to what kind of acid/base imbalance? |
Metabolic acidosis |
|
Overuse of diuretics leads to what kind of acid/base imbalance? |
Loss of hydrogen ions leading to metabolic alkalosis |
|
Panic attacks leads to what kind of acid/base imbalance? |
Respiratory alkalosis |
|
COPD leads to what kind of acid/base imbalance? |
Respiratory acidosis (compensated) pH - decreased PCO2 - increased HCO3- - inceased |
|
What is the inheritance pattern of cystic fibrosis? |
Autosomal recessive |
|
Failure of neutrophils to turn blue on a nitroblue tetrazolium test indicates what disease? |
Chronic Granulomatous Disease |
|
Neutrophils in Chronic Granulomatous Disease are unable to kill what and why? |
Catalase producing organisms because normally bacteria will kill themselves due to their own production of H2O2. If the organism can break down its own H2O2 then it can survive. The body can not make any H2O2 since NADPH oxidase does not work. |
|
Cheyne-stokes breathing is commonly seen in what? |
advanced congestive heart failure |
|
Stimulation of vagus nerve would do what to the lungs (2)? |
Bronchoconstriction and increased mucus production |
|
Cystic fibrosis is caused by what genetic defect? What does it affect as far as going from gene to protein? |
3-base pair deletion of CFTR (cystic fibrosis transmembrane conductance regulator). This affects post-translational processing. |
|
Phospholipid content of amniocentesis is used to check for what? |
Fetal Lung development (measuring phosaphatidylcholine) |
|
Bronchioalveolar lavage of patients with sarcoidosis show elevation in what cell type? |
CD4+ lymphocytes |
|
Bronchioalveolar lavage of patients with lung transplants showing rejection would show elevation in what cell type? |
CD8+ lymphocytes |
|
Structure of MHC Class I |
Heavy chain and B2 microglobulin |
|
Structure of MHC Class II |
Alpha and beta polypeptide chains |
|
Which cells are MHC Class I molecules expressed? |
All cells |
|
Which cells are MHC Class II molecules expressed? |
Antigen Presenting Cells |
|
Describe V/Q in base vs apex |
Higher in apex lower in base |
|
What is used as a marker for mast cell activation (anaphylaxis)? |
Tryptase |
|
Symptoms of Theophylline overdose? (3) |
abdominal pain, vomiting, seizures |
|
Behcet syndrome has what 3 parts? |
Apthous ulcers, genital ulcers, and uveitis |
|
Pathophysiology of behcet's syndrome |
Immune complex vasculitis involving small vessels |
|
Most feared complication of mumps? |
Orchitis leading to sterility |
|
Benign cystic tumor with abundant lymphocytes and germinal centers found in salivary gland |
Warthin tumor |
|
Congenital defect resulting in a connection between the esophagus and trachea |
Tracheoesophageal fistula |
|
Thin protrusion of esophageal mucosa, most often in the upper esophagus |
Esophageal web |
|
What are the components of Plummer-Vinson syndrome? |
Severe iron deficiency anemia, esophageal web, beefy-red tongue due to atrophic glossitis |
|
Outpouching of pharyngeal mucosa through acquired defect in the muscular wall of the esophagus |
Zenker Diverticulum |
|
Longitudinal laceration of mucosa at the GE junction |
Mallory-Weiss syndrome |
|
What is the cause of Mallory-Weiss syndrome? and what 2 populations is it seen in? |
Severe vomiting due to bulimia or alcoholism |
|
rupture of esophagus leading to air in mediastinum and subcutaneoues emphysema |
Boerhaave syndrome |
|
Mallory-Weiss syndrome has a risk of what? |
Boerhaave syndrome |
|
Dilated submucosal veins in the lower esophagus |
Esophageal varices |
|
Esophageal varices arise secondary to what? |
portal HTN |
|
Painless hematemesis is found in what? Painful hematemesis is found in what? |
Painless = esophageal varices Painful = Mallory-Weiss syndrome |
|
Most common cause of death in cirrhosis? |
Esophageal varices that rupture |
|
Disordered esophageal motility with inability to relax lower esophageal sphincter |
Achalasia |
|
Achalasia is due to damage of what? |
Ganglion cells in MYENTERIC plexus |
|
Achalsia can be due to infection by what bacteria, and what is the name of the disease? |
Trypanosoma cruzi = Chagas disease |
|
Reflux of acid from the stomach due to reduced LES tone |
GERD |
|
Metaplasia of the lower esophageal mucosa from stratified squamous epithelium to nonciliated columnar epithelium with goblet cells |
Barrett Esophagus |
|
Two types of esophageal carcinoma |
Adenocarcinoma and squamous cell carcinoma |
|
Most common esophageal cancer worldwide |
squamous cell carcinoma |
|
Most common esophageal cancer in the west |
adenocarcinoma |
|
(1)Upper 1/3, (2)Middle 1/3, and (2)Lower 1/3 of esophageal cancers involve what lymph nodes? |
upper = cervical middle = mediastinal and tracehobroncial nodes lower = celiac and gastric |
|
Congenital malformation of the anterior abdominal wall leading to exposure of abdominal contents |
Gastroschisis |
|
Persistent herniation of bowel into umbilical cord |
Omphalocele |
|
Congenital hypertrophy of pyloric smooth muscle |
Pyloric Stenosis |
|
Acidic damage to the stomach mucosa |
Acute Gastritis |
|
Projectile nonbilious vomiting indicates? |
Pyloric stenosis |
|
Which cells in the stomach produce mucous? |
foveolar cells |
|
What is a cushing ulcer? |
Acute gastritis induced ulcer due to increased stimulation of vagus nerve to release acetylcholine which leads to increased acid production |
|
What are the 3 receptors on parietal cells that can induce it to secrete acid? |
Acetylcholine Gastrin Histamine |
|
What are the two types of chronic gastritis? |
Chronic Autoimmune gastritis Chronic H pylori gastritis |
|
Autoimmune destruction of gastric parietal cells leads to? |
Chronic gastritis |
|
What regions are gastric parietal cells located in the stomach? |
Fundus and Body NOT Cardia and Antrum |
|
What region is Chronic H pylori gastritis normally affect? |
Antrum NOT fundus, body, or cardia |
|
Solitary mucosal ulcer that occurs in the duodenum 90% of the time and in the distal stomach 10% of the time |
Peptic ulcer disease |
|
Duodenal or gastric ulcers; pain improves with meals? |
Duodenal |
|
Malignant proliferation of surface epithelial cells in the stomach |
Gastric carcinoma |
|
Two types of gastric carcinoma |
intestinal type diffuse type |
|
Gastric carcinoma which presents as large, irregular ulcer with heaped up margins |
intestinal type |
|
Gastric carcinoma which is characterized by signet ring cells that infiltrate the gastric wall |
Diffuse type |
|
Blood type A is associated with what cancer? |
Intestinal Type of gastric carcinoma |
|
describe a signet ring cell? |
Nucleus pushed off to the edge due to production of mucin |
|
Leser-Trelat sign can indicate what? |
Gastric carcinoma |
|
Gastric carcinoma normally spreads to which node? |
Left supraclavicular node (virchow node) |
|
Which node is Virchow node? |
Left supraclavicular node |
|
Distant metastasis of gastric carcinoma most commonly involves what organ? |
Liver |
|
Intestinal type of gastric carcinoma classically spreads where? |
Preumbilical region (Sister Mary Joseph nodule) |
|
Diffuse type of gastric carcinoma classically spreads where? |
Bilateral ovaries (Krukenberg tumor) |
|
What disease has translocation at 12,21 |
ALL (kids) |
|
What disease has translocation at 9,22 |
ALL (adults) and CML |
|
What disease has translocation at 15,17 |
Acute Promyelocytic Leukemia |
|
What disease has translocation at 14,18 |
Follicular lymphoma |
|
What disease has translocation at 11,14 |
Mantle Cell Lymphoma |
|
What disease has translocation at 8,14 |
Burkitt Lymphoma |
|
Congenital failure of small bowel to canalize |
Duodenal atresia |
|
What is duodenal atresia associated with? |
Down syndrome |
|
Double-bubble sign indicates what pathology? |
Duodenal atresia |
|
Outpouching of all three layers of bowel wall |
Meckel Diverticulum |
|
This pathology occurs due to failure of the vitelline duct to involute |
Meckel diverticulum |
|
What are the (4) Rule of 2's and what does it apply to? |
Meckel Diverticulum Seen in 2% of population Usually 2 inches long Within 2 feet of ileocecal valve Present during first 2 years of life |
|
Twisting of bowel along its mesentery |
Volvulus |
|
Telescoping of proximal segment of bowel forward into distal segment |
Intussusception |
|
Most common cause for intussusception in adults |
tumor |
|
Most common cause for intussusception in children |
Lymphoid hyperplasia |
|
Immune-mediate damage of small bowel villi due to gluten exposure |
Celiac disease |
|
What is the most pathogenic component of gluten? |
Gliadin |
|
What is the process by which Gliadin causes damage to villi? |
Gliadin is deamidated by tTg (tissue transgutaminase), it is then presented to APC via MHC Class II. Helper T cells mediate tissue damage |
|
Dermatitis herpetiformis is associated with what GI pathology? |
Celiac disease - small, herpes-like vesicles may arise on skin due to IgA deposition at the tips of dermal papillae; resolve with gluten-free diet |
|
What laboratory findings are there for celiac disease? |
IgA antibodies against endomysium, tTG, or gliadin |
|
In celiac disease which portion of the small intestine is most involved? |
Duodenum |
|
If there is refractory disease after continuing a gluten free diet when dx with celiac what should you suspect (2)? |
Small bowel carcinoma and T-cell lymphoma (eneteropathy associated T-cell lymphoma) |
|
Damage to small bowel villi due to an unknown organism resulting in malabsorption |
Tropical sprue |
|
In tropical sprue which portion of the small intestine is involved? |
Jejunum and Ileum |
|
Where is folic acid absorbed? |
Jejunum |
|
Where is Vit B12 absorbed? |
Ileum |
|
What is whipple disease? |
Systemic tissue damage characterized by macrophages loaded with Tropheryma Whippelii organisms |
|
Two key findings in whipple disease? |
fat malabsorption and steatorrhea |
|
Abetalipoproteinemia has what inheritance pattern and is a deficiency in what two proteins |
Autosomal recessive deficiency of apolipoprotein B-48 and B-100 |
|
Patient has absent plasma VLDL and LDL, what is the dx and what is the pathophysiology? |
Abetalipoproteinemia - VLDL and LDL require apolipoprotein B-100 |
|
Abscence of apolipoprotein B-48 indicate what disease and has what feature due to this absence? |
Abetalipoproteinemia - absence of B-48 leads to defective chylomicron formation |
|
Carcinoid tumor in the gut most commonly arises where? |
Small bowel |
|
Carcinoid tumor of small bowel secretes what? which is metabolized where? |
Serotonin metabolized in liver into 5-HIAA |
|
What are the 3 clinical features of carcinoid syndrome? |
bronchospasm, diarrhea, and flushing of skin |
|
What is the pathophysiology for carcinoid syndrome from a carcinoid tumor in the small gut? |
Metastasis of carcinoid tumor to liver allows serotonin to bypass liver metabolism leading to excess serotonin in the systemic circulation |
|
What is carcinoid heart disease? |
Right-sided valvular fibrosis leading to tricuspid regurgitation and pulmonary valve stenosis. |
|
Why do you not get left sided valvular fibrosis in carcinoid heart disease? |
Serotonin gets metabolized by MAO in the lung |
|
What quadrant is there pain in acute appendicitis? |
right lower quadrant (McBurney) point |
|
Chronic, relapsing inflammation of bowel |
Inflammatory Bowel Disease |
|
Which IBD has full thickness inflammation with knife-like fissures? |
Crohn disease |
|
Which IBD has mucosal and submucosal ulcers? |
Ulcerative colitis |
|
Which IBD skips around and which one is continuous? |
Ulcerative colitis is continuous, Crohn's skips around |
|
Which IBD has left lower quadrant pain? |
Ulcerative colitis |
|
Which IBD has right lower quad pain? |
Crohn disease |
|
Which IBD has histology with crypt abscesses with neutrophils? |
Ulcerative Colitis |
|
Which IBD has loss of haustra "lead pipe" sign on imaging? |
Ulcerative colitis |
|
Which IBD has cobblestone mucosa? |
Crohn disease |
|
Which IBD has pseudopolyps? |
Ulcerative colitis |
|
Which IBD has toxic megacolon as a complicatoin? |
Ulcerative colitis |
|
Which IBD has p-ANCA positivity? |
Ulcerative colitis |
|
Which IBD is protected by smoking? |
Ulcerative colitis |
|
Which IBD has increased risk due to smoking? |
Crohn disease |
|
Which IBD has lymphoid aggregates with granulomas? |
Crohn disease |
|
Which IBD has string-sign on imaging? |
Crohn disease |
|
Which IBD has calcium oxalate neprholithiasis? |
Crohn disease |
|
Defective relaxation and peristalsis of rectum and distal sigmoid colon |
Hirschsprung disease |
|
What is hirschsprung disease associated with? |
Down syndrome |
|
Rectal suction biopsy reveals lack of ganglion cells what disease is this? |
Hirschsprung disease |
|
Outpouching of mucosa and submucosa through the muscularis propria |
Colonic diverticula |
|
Acquired malformation of mucosal and submucosal capillary beds |
Angiodysplasia |
|
High stress on the left lower quadrant leads to what? |
Colonic diverticula |
|
High stress on the right colon leads to what? |
Angiodysplasia |
|
If a patient has stool or air in urine what is the reason and what is the disease called? |
Inflamed diverticulum ruptures and attaches to a local structure. Colovesicular fistula due to colonic diverticula. |
|
What is the inheritance pattern for hereditary hemorrhagic telangiectasia? |
Autosomal dominant |
|
Ischemic damage to the colon usually at the splenic flexure |
Ischemic colitis |
|
This GI disease presents with postprandial pain and weight loss |
Ischemic colitis |
|
Relapsing abdominal pain with bloating, flatulence, and change in bowel habits (diarrhea or constipation) |
Irritable bowel syndrome |
|
Raised protrusion of colonic mucosa |
Colonic polyps |
|
Two most common types of colonic polyps |
Hyperplastic polyps and adenomatous polyps |
|
This type of polyp are due to hyperplasia of glands; classically show a serrated appearance on microscopy |
Hyperplastic polyp |
|
Which of the two most common colonic polyps is benign not malignant, and which is benign but premalignant? |
Hyperplastic polyp is benign Adenomatous polyp is premalignant |
|
What chromosome is APC located on? |
Chromosome 5 |
|
What is the adenoma-carcinoma sequence (3 steps) |
1.APC (adenomatous polyposis coli gene) mutation leads to increased risk for formation of polyps 2. K-ras mutation leads to formation of polyps 3. p53 mutation and increased expression of COX allow for progression to carcinoma |
|
What can impede the progression of adenoma to carcinoma in colonic polyps? |
Aspirin due to the inhibition of COX |
|
What 3 characteristics of a colon polyp biopsy indicates an increased risk for progression from adenoma to carcinoma? |
Size >2cm sessile growth as opposed to pedunculated villous histology as opposed to tubular |
|
Autosomal dominant disorder characterized by 100s to 100s of adenomatous colonic polyps |
Familial adenomatous polyposis |
|
What is the inheritance pattern for familial adenomatous polyposis? |
Autosomal dominant |
|
What is familial adenomatous polyposis with fibromatosis and osteomas? |
Gardner syndrome |
|
What is familial adenomatous polyposis with CNS tumors? |
Turcot syndrome |
|
Sporadic hamartomatous (benign) polyp that arises in children |
Juvenile polyp |
|
Hamartomatous (benign) polyps throughout GI tract with mucocutaneous hyperpigmentation on lips, oral mucosa, and genital skin? |
Peutz-Jeghers syndrome |
|
What is the inheritance pattern for Peutz-Jeghers syndrome? |
Autosomal dominant |
|
Peutz-Jeghers syndrome has increased risk for what 3 cancers? |
colorectal breast GYN |
|
What is the 3rd most common site of cancer and the 3rd most common cause of cancer-related death? |
Colorectal carcinoma |
|
What are the two pathways that colorectal carcinoma can arise? |
Adenoma-carcinoma sequence and microsatellite instability (MSI) |
|
Hereditary nonpolyposis colorectal carcinoma (HNPCC) is due to inherited mutations in what? |
DNA mismatch repair enzymes |
|
Inherited mutations in DNA mismatch repair enzymes can lead to what hereditary GI disorder? |
Hereditary nonpolyposis colorectal carcinoma (HNPCC) |
|
HNPCC has increased risk for what 3 cancers? |
Colorectal (most common) Ovarian Endometrial carcinoma |
|
Which side do napkin-ring lesions for colorectal carcinoma usually arise? |
left side |
|
Which side do raised lesion for colorectal carcinoma usually arise? |
right side |
|
Colonic carcinoma is associated with an increased risk for what kind if infection? |
Streptococcus bovis endocarditis |
|
Left sided colorectal carcinoma is associated with what molecular pathway? |
Left Adenoma-carcinoma pathway Right MSI pathway |
|
Most common site for colorectal carcinoma metastasis? |
Liver |
|
What serum tumor marker is useful for assessing tx response and detecting recurrence of colorectal carcinoma |
CEA |
|
CEA is useful for assessing tx response and detecting recurrence but not useful for what? |
Not useful for screening |
|
Where is Iron (Fe2+) absorbed? |
Duodenum |
|
Developmental malformation in which the pancreas forms a ring around the duodenum; risk of duodenal obstruction |
Annular pancreas |
|
Annular pancreas has an increased risk for what? |
Duodenal obstruction |
|
Inflammation and hemorrhage of pancreas |
Acute pancreatitis |
|
What is the first enzyme to get activated in acute pancreatitis? |
Trypsin |
|
Alcohol and gall stones are the most common cause for what? |
Acute pancreatitis - alcohol constricts sphincter of oddi and gall stones create a blockage |
|
Fibrosis of pancreatic pancrenchyma; secondary to recurrent acute pancreatitis |
Chronic pancreatitis |
|
Most commonly due to alcohol and cystic fibrosis; many cases are idiopathic |
Chronic pancreatitis |
|
Alcohol and cystic fibrosis are the most common cause of what? |
Chronic pancreatitis |
|
Adenocarcinoma arising from pancreatic ducts |
Pancreatic carcinoma |
|
What are the two major risk factors for pancreatic carcinoma? |
Smoking and chronic pancreatitis |
|
If a thin elderly female recently dx with diabetes what should you think of? |
DDX should include pancreatic carcinoma |
|
What is the serum tumor marker for pancreatic carcinoma? |
CA 19-9 |
|
Failure to form or early destruction of extrahepatic biliary tree |
Biliary atresia |
|
Solid, round stones in the gall bladder |
Cholelithiasis (Gall stones) |
|
Cholelithiasis can occur due to precipitation of what two things? |
Cholesterol or bilirubin |
|
Important side effect of cholestyramine and MOA for the side-effect |
Gallstones due to decreased solubility of bilirubin. Bile acids are bound by cholestyramine leading to decreased solubility and formation of gall stones. |
|
Which gall stone is radiolucent? radiopaque? |
Cholesterol stone is radiolucent, bilirubin stone is radiopaque |
|
Waxing and waning right upper quadrant pain is usually due to? |
Biliary colic |
|
Biliary colic pathophysiology for pain |
This is due to gallbladder contracting against a stone lodged in the cystic duct |
|
Acute inflammation of the gallbladder wall |
Acute cholecystitis |
|
Right upper quadrant pain, radiating to the right scapula indicates what? |
Acute cholecystitis |
|
Chronic inflammation of the gallbladder |
Chronic cholecystitis |
|
Rokitansky-Aschoff sinus is associated with what? and what is it? |
Chronic cholecystitis - these sinuses are herniations of the gallbladder mucosa into the muscular wall. Normally you should not have mucosa into the smooth muscle. |
|
Porcelain gallbladder is associated with what? |
Chronic cholecystitis |
|
This presents with vague right upper quadrant pain, especially after eating |
Chronic cholecystitis |
|
Bacterial infection of bile ducts |
Ascending cholangitis |
|
Gallstones enters and obstructs small bowel |
Gallstone ileus |
|
What is gallstone ileus due to? |
Cholecystitis with fistula formation between gallbladder and small bowel |
|
Adenocarcinoma arising from glandular epithelium that lines the gallbladder wall |
Gallbladder carcinoma |
|
Elderly woman presents with cholecystitis, what does this suggest? |
Gallbladder carcinoma |
|
What is scleral icterus? |
yellowing of the eye |
|
How does extravascular hemolysis or ineffective erythropoiesis cause jaundice? |
High levels of UCB overwhelm the conjugating ability of the liver |
|
Dark urine in extravascular hemolysis is due to what? what is it not due to? |
Increased urobilinogen NOT UCB (UCB is not water soluble) |
|
Extravascular hemolysis or ineffective erytrhopoiesis has an increased risk for what hepatic disorder? |
pigmented bilirubin gallstones |
|
How does physiologic jaundice of the newborn cause jaundice? |
Newborn liver has transiently low UGT activity |
|
What enzyme conjugates unconjugated bilirubin? |
Uridine Glucuronyl Transferase (UGT) |
|
What is kernicterus associated with and what is the pathophysiology of it? |
Kernicterus is associated with Physiologic jaundice of the newborn and Crigler-Najjar syndrome. UCB is fat soluble and can deposit in the basal ganglia leading to neurological deficits and death. |
|
How does phototherapy work for physiologic jaundice of the newborn? |
It makes the UCB water soluble but does NOT conjugate the UCB |
|
How does Gilbert syndrome cause jaundice? |
Midly low UGT activity. Usually enough UGT not to cause jaundice but at times of high stress will cause jaundice |
|
What is the inheritance pattern for gilbert syndrome? |
Autosomal recessive |
|
How does Crigler-Najjar syndrome cause jaundice? |
Complete absence of UGT, usually fatal |
|
Why is Criger-Najjar syndrome usually fatal? |
Kernicterus |
|
What are the two syndromes with disruption of UGT? |
Gilbert Syndrome - mildly low Criger-Najjar syndrome - complete absence |
|
How does Dubin-Johnson syndrome cause jaundice? |
Deficiency of bilirubin canalicular transport protein |
|
What is the inheritance pattern for Dubin-Johnson syndrome? |
Autosomal recessive |
|
Which disease that causes jaundice has a dark liver? |
Dubin-Johnson syndrome |
|
What syndrome is similar to Dubin-Johnson syndrome but does not have the dark liver? |
Rotor syndrome |
|
How does biliary tract obstruction cause jaundice? |
Buildup of CB in gallbladder leaks out into the blood |
|
Why is urine dark in biliary tract obstruction? |
Due to conjugated bilirubin |
|
How does viral hepatitis cause jaundice? |
Inflammation disrupts hepatocytes and small bile ductules leading to increase in CB and UCB |
|
Why is urine dark in viral hepatitis? |
Due to conjugated bilirubin |
|
Which disease that causes jaundice has pruritis and why does it have pruritis? |
Biliary tract obstruction - pruritis is due to increased plasma bile acids |
|
Which disease that causes jaundice has xanthomas and why does it have xanthomas? |
Biliary tract obstruction - hypercholestermemia leads to xanthomas |
|
Which disease that causes jaundice has steatorrhea with malabsorption of fat-soluble vitamins? |
Biliary tract obstruction |
|
Viral hepatitis is usually due to hepatitis virus but what other two viruses can cause it? |
EBV and CMV |
|
Viral hepatitis has elevated liver enzymes which one is higher than the other? |
ALT > AST |
|
Alcohol-related liver disease has elevated liver enzymes which one is higher than the other? |
AST > ALT |
|
Which Hepatitis virus has fecal-oral transmission? |
HAV and HEV |
|
Which hepatitis is commonly acquired by travelers? |
HAV |
|
Which hepatitis is commonly acquired from contaminated water or undercooked seafood? |
HEV |
|
Which hepatitis is associated with pregnant woman with fulminant hepatitis (liver failure with massive liver necrosis)? |
HEV infection |
|
Which hepatitis has only an acute state and no chronic state? |
HAV and HEV |
|
Which hepatitis is transferred parenterally? |
HBV and HCV |
|
What marker must be present for 6 months to indicate chronic hepatitis B? |
HBsAG (antigen) |
|
Presence of what indicates that you have beat hepatitis B? |
HBsAB (antibody) |
|
What marker indicates that HBV is infectious? |
HBeAG (envelope antigen) |
|
Which hepatitis often progresses to chronic phase, which hepatitis 20% of the time progresses to chronic phase? |
HCV - most of the time HBV - 20% of time |
|
What marker indicates HCV infection and indicates chronic disease if persistent? |
HCV-RNA |
|
Which hepatitis virus needs another hepatitis virus to infect? |
HDV |
|
What is more severe? Superinfection of HBV/HDV or co-infection of HBV/HDV? |
Superinfection |
|
End-stage liver damage characterized by disruption of the normal hepatic parenchyma by bands of fibrosis and regenerative nodules of hepatocytes |
Cirrhosis |
|
Fibrosis of liver is mediated by what cell which secretes what cytokine? |
Stellate cells secreting TGF-B |
|
What are mallory bodies and what is it associated with? |
Mallory bodies are damaged intermediate filaments within hepatocytes and is associated with alcoholic hepatitis |
|
Nonalcoholic fatty liver disease has increased liver enzymes, which is higher than the other? |
ALT > AST |
|
What is nonalcoholic fatty liver disease associated with? |
obesity |
|
Primary Hemochromatosis has what kind of inheritance pattern? |
Autosomal recessive |
|
Primary hemochromatosis is due to a mutation in which gene? |
HFE gene |
|
Bronze skin and bronze diabetes is associated with what? |
Hemochromatosis |
|
For hemochromatosis hepatocytes are brown, but you need to distinguish the iron from what? what stain do you use to do this? |
Lipofuscin caused by normal wear and tear, use prussian blue stain to stain the iron. |
|
Autosomal recessive defect in ATP-mediated copper transport |
Wilson disease |
|
Wilson disease has what inheritance pattern? |
Autosomal recessive |
|
Autoimmune granulomatous destruction of intrahepatic bile ducts |
Primary biliary cirrhosis |
|
Primary biliary cirrhosis, etiology is unknown but something is present, what is it? |
antimitochondrial antibody |
|
Inflammation and fibrosis of intrahepatic and extrahepatic bile ducts |
Primary sclerosing cholangitis |
|
Periductal fibrosis with an "onion-skin" appearance on histology indicates what hepatic disease? |
Primary sclerosing cholangitis |
|
What hepatic disorder is associated with ulcerative colitis? |
Primary sclerosing cholangitis |
|
What hepatic disorder is p-ANCA positive? |
Primary sclerosing cholangitis |
|
What is reye syndrome? |
Fulminant liver failure and encepholopathy in children with viral illness who take aspirin |
|
Benign tumor of hepatocytes |
Hepatic adenoma |
|
Reye syndrome is likely related to what kind of damage? |
mitochondrial damage of hepatocytes |
|
Hepatic adenoma is associated with what drug? |
oral contraceptive |
|
Malignant tumor of hepatocytes |
Heptaocellular carcinoma |
|
Alfatoxins derived from Aspergillus is associated with what cancer? what mutation does it cause? |
Hepatocellular carcinoma - p53 mutation |
|
What is the serum tumor marker for hepatocellular carcinoma? |
alpha-fetoprotein |
|
Which glucose transporter is responsible for transportation to the blood? |
GLUT-2 |
|
Rate limiting enzyme for bile synthesis |
Cholesterol 7a-hydroxylase |
|
What is a curling ulcer? |
Ulcer that forms when there is decreased plasma volume (burn victims) which leads to sloughing of gastric mucosa |
|
Which gastric cancer is associated with nitrosamines (Smoked food) |
Intestinal |
|
What is Charcot triad of cholangitis? |
Jaundice, Fever, RUQ pain |
|
Drugs ending in -dine have what MOA? |
H2 blocker |
|
Drugs ending in -prazole have what MOA? |
Proton pump inhibitor |
|
MOA of Misoprostol |
PGE1 analog, increases gastric mucosa barrier |
|
MOA of Octreotide |
Long-acting somatostatin analong |
|
MOA of Infliximab and what is it used for? |
Monoclonal antibody to TNF-a Chron disase, ulcerative colitis, RA, psoriasis, ankylosing spondylitis |
|
MOA of Sulfasalazine |
combo of sulfapyridine (antibacterial) and 5-ASA (anti-inflammatory). Activated by colonic bacteria |
|
MOA of Ondansetron and what is it used for? |
5-HT3 blocker; decrease vagal stimulation. Powerful central acting anti-emetic. |
|
MOA of Metoclopramide and what is it used for? |
D2 receptor antagonist. Increase resting tone, contractility, LES tone, motility. Used for GERD, anti-emetic, gastric emptying. |
|
Drugs ending in -odipine have what MOA? |
Calcium channel blockers that block L-type calcium channels, thereby reducing cardiac muscle contractility. |
|
MOA of hydralazine |
increase cGMP leading to smooth muscle relaxation. |
|
What two drugs are used in a hypertensive emergency and what is their MOA? |
Nitroprusside - increase cGMP via direct release of NO. Fenoldopam - Dopamine D1 receptor agonist causing vasodilation. |
|
MOA of nitroglycerin |
Vasodilate by increasing NO in vascular smooth muscle. |
|
MOA of digoxin |
Direct inhibition of Na/K atapase leads to indirect inhibition of Na/Ca exchanger/antiport. Increase in Ca leads to positive inotropy (cardiac contraction). Stimulates vagus nerve to decrease HR. |
|
MOA of drugs ending in -en/-ine or en/-ate? |
1st gen H1 blockers (diphenhydramine, dimenhydrinate, chloropheniramine) |
|
MOA of drugs ending in -adine? |
2nd gen H1 blockers (Loratidine, fexofenadine, desloratadine, cetirizine |
|
What does guaifenesin? |
Expectorant - thins respiratory secretions, does not suppress cough reflex |
|
What does N-acetylcysteine do? |
Mucolytic - can loosen mucous plugs in CF patients |
|
MOA of dextromethorphan? |
Antitussive (antagonizes NMDA glutamate receptor) |
|
MOA of pseudoephedrine and phenylephrine? |
alpha agonist |
|
MOA of albuterol? what is it used for? |
B2 agonist, asthma |
|
MOA of salmeterol? what is it used for? |
B2 agonist, asthma |
|
MOA of formoterol? what is it used for? |
B2 agonist, asthma |
|
MOA of theophylline and what is it used for? |
causes bronchodilation by inhibiting phosphodiesterase which increase cAMP due to decrease in cAMP hydrolysis. Asthma. |
|
MOA of ipratropium and what is it used for? |
competitive block of muscarinic receptors, preventing bronchoconstriction. Asthma. |
|
MOA beclomethasone and what is it used for? |
Inhibit synthesis of virtually all cytokines. Inactivate NF-kB, the transcription factor that induces production of TNF-a and other inflammatory agents. Asthma. |
|
MOA of fluticasone and what is it used for? |
Inhibit synthesis of virtually all cytokines. Inactivate NF-kB, the transcription factor that induces production of TNF-a and other inflammatory agents. Asthma. |
|
MOA of drugs ending in -lukast (Montelukast, Zafrilukast) and what is it used for? |
block leukotriene receptors. Especially good for aspirin-induced asthma. Asthma |
|
MOA of Zileuton and what is it used for? |
5-lipoxygenase pathway inhibitor blocks conversion of arachidonic acid to leukotrienes. Asthma |
|
MOA of omalizumab and what is it used for? |
Monoclonal anti-IgE antibody. Binds mostly unbound serum IgE. Asthma |
|
MOA of methacoline and what is it used for? |
Muscarinic receptor agonist. Used to dx asthma |
|
Drugs ending in -navir have what MOA and treat what? |
Protease inhibitor, specifically inhbits HIV-1 protease (pol gene). Protease inhibitors prevent maturation of new viruses. |
|
MOA of Raltegravir |
Inhibits HIV genome integration to host cell chromosome by reversibly inhibiting HIV integrase |
|
MOA of Enfuviritide |
Binds gp41, inhibiting viral entry |
|
MOA of Maraviroc |
Binds CCR-5 on surface of T cells/monocytes, inhibiting interaction with gp120 |
|
MOA of Zidovudine |
Nucleoside Reverse Transcriptase Inhibitor |
|
IL-8 is responsible for what? |
Released by macrophages to recruit neutrophils |
|
IL-3 is responsible for what? |
Stimulating growth and differentiation of stem cells in bone marrow. |
|
What do each of the blotting techniques detect?North, south, west, southwest? |
North = RNA South = DNA West = Protein Southwest = DNA binding protein |
|
MOA of Ribavarin? |
Inhibits synthesis of guanine nucleotides by competitively inhibiting inosine monophosphate dehydrogenase |
|
Cricopharyngeal motor dysfunction can lead to what? |
Zenker diverticulum |
|
Two effects of a VIPoma and how do you treat VIPoma? |
diarrhea and inhibition of gastric. Tx with somatostatin |
|
MOA of diphenoxylate and what is it used for? |
opiate anti-diarrheal that slows down motility |
|
What is a liver specific enzyme that can use break down products of triglyceride? |
Glycerol kinase |
|
What two compounds are elevated in lead poisoning? |
delta-aminolevulinic acid and pyroxidal phosphate |
|
Where are lipids digested? where are they absorbed? |
Duodenum digests, jejenum absorbs |
|
Which portion of the GI tracts is ALWAYS affected in hirschprung disease? |
Rectum |
|
Where are Brunner's glands located and what do they secrete? |
Alkaline mucous |
|
Where are peyer's patches found in the small intestine? |
Ileum |
|
What triggers hepatocellular carcinoma when HBV infects the liver? |
Integration of the viral DNA with the host DNA. |
|
Conjoined kidneys usually connected at the lower pole. |
Horseshoe kidney |
|
Where is the horseshoe kidney located, what does it get stuck on? |
Located in lower abdomen it gets stuck on inferior mesenteric artery on its ascent. |
|
Absent kidney formation |
Renal agenisis |
|
Describe the Potter sequence? |
Bilateral renal agenesis leads to -oligohydraminos -lung hypolplasia -flat face with low set ears -development defects of extremities |
|
Noninherited, congenital malformation of renal parenchyma characterized by cysts and abnormal tissue. |
Dysplastic kidney |
|
Inherited defect leading to bilateral enlarged kidneys with cysts in the renal cortex and medulla |
Polycystic Kidney Disease |
|
Which cystic kidney disease is unilateral and which is bilateral? |
Dysplastic kidney - usually unilateral Polycystic Kidney disease - bilateral |
|
Autosomal recessive or dominant PKD presents in infants? |
Recessive |
|
Autosomal recessive or dominant PKD presents with worsening renal failure and HTN? |
Recessive and Dominant |
|
Autosomal recessive or dominant PKD may present with potter sequence? |
Recessive |
|
Autosomal recessive or dominant PKD associated with congenital hepatic fibrosis and cysts? |
Recessive |
|
Autosomal recessive or dominant PKD presents in young adults? |
Dominant |
|
Autosomal dominant PKD is due to either of what two mutations? |
APKD1 or APKD2 |
|
Autosomal recessive or dominant PKD presents with hematuria? |
Dominant |
|
Autosomal recessive or dominant PKD associated with berry aneurysm |
Dominant |
|
Autosomal recessive or dominant PKD associated with mitral valve prolapse and hepatic cysts? |
Dominant |
|
Autosomal dominant PKD presents with what three cysts? |
Cysts in Kidney Cysts in Brain (berry aneurysm) Cysts in Liver |
|
Medullary cystic kidney disease has what inheritance pattern? |
Autosomal Dominant |
|
Cysts in medullary collecting ducts |
Medullary cystic kidney disease |
|
Parenchymal fibrosis results in SHRUNKEN kidneys and worsening renal failure |
Medullary cystic kidney disease |
|
Hallmark of Acute renal failure? |
Azotemia often with oliguria |
|
Acute renal failure due to decreased blood flow to kidney |
Prerenal azotemia |
|
Acute renal failure due to obstruction of urinary tract downstream from kidney |
Postrenal azotemia |
|
Prerenal or postrenal azotemia: BUN:Cr ratio >15? |
Prerenal or Postrenal (early) - BUN is reabsorbed due to aldosterone secretion while creatinine can not be reabsorbed |
|
Prerenal or postrenal azotemia: BUN:Cr ratio <15? |
Postrenal (late) - can not reabsorb BUN because the renal tubles or no longer intact? |
|
Prerenal or postrenal: FENa < 1% |
Fractional Excretion of sodium < 1 percent in Prerenal |
|
Prerenal or postrenal: FENa >2% |
Fractional excretion of sodium is >2% in postrenal (late) azotemia |
|
Prerenal or postrenal: Urine osmalality > 500 mOsm/kg? |
Prerenal or postrenal (early) |
|
Prerenal or postrenal: Urine osmalality < 500 mOsm/kg? |
Postrenal (late) - inability to concentrate urine |
|
Injury and necrosis of tubular epithelial cells; most common cause of acute renal failure |
Acute tubular necrosis |
|
Pathophysiology of acute tubular necrosis |
Necrotic epithelial cells of tubules plug tubules; obstruction decreases GFR |
|
What renal disease has brown, granular casts that are seen in urine? |
Acute tubular necrosis |
|
Is BUN:Cr < or > 15 in acute tubular necrosis? |
BUN:CR is < 15 because of inability to reabsorb BUN |
|
Is FENa <1 or >2 in acute tubular necrosis? |
Fractional Excretion of Na (FENa) is >2 because of decreased reabsorption of sodium |
|
Is urine osm < or > 500 in acute tubular necrosis? |
Inability to concentrate urine leads to urine osm to be <500. |
|
What are the two major etiologies for acute tubular necrosis? |
Ischemic and nephrotoxic |
|
Prerenal azotemia can progress to what disease? |
Acute tubular necrosis |
|
Which drug class can cause acute tubular necrosis? |
Aminoglycosides |
|
Crush injury can lead to what? |
Myoglobinuria which leads to acute tubular necrosis |
|
Ingesting ethylene glycol can lead to what? |
Acute tubular necrosis |
|
Acute tubular necrosis leads to what acid/base imbalance? |
Metabolic acidosis |
|
Drug-induced hypersensitivity involving the interstitium and tubules of kidneys |
Acute interstitial nephritis |
|
What three drugs can cause interstitial nephritis? |
NSAIDS, PCN, and diuretics |
|
What is pathognomonic for acute interstitial nephritis? |
Eosinophils seen in urine |
|
Presents as oliguria, fever and rash days to weeks after starting a drug |
Acute interstitial nephritis |
|
Necrosis of renal papillae |
Renal papillary necrosis |
|
Glomerular disorders with proteinuria >3.5g/day characterizes what group of disorders? |
Nephrotic syndrome |
|
Nephrotic syndromes result in 2 hypos and 3 hypers |
Hypoalubminemia - pitting edema Hypogammaglobulinemia - increases risk of infection Hypercoagulable state - loss of antithrombin III Hyperlipidemia Hypercholesterolemia - may lead to fatty casts in urine |
|
Which nephrotic syndrome is associated with Hodgkin lymphoma? |
Minimal change disease |
|
What is the most common nephrotic syndrome in children? |
Minimal change disease |
|
Which nephrotic syndrome has effacement of foot processes that is mediated by cytokines? |
Minimal change disease mediated by cytokines that are released from reed-sternberg cells of hodgkin lymphoma |
|
Which nephrotic syndrome has normal glomeruli on H&E stain? |
Minimal change disease |
|
Which nephrotic syndrome has no immune complexes so negative immunofluorescence. (2) |
Minimal change disease and Focal segmenting glomerulosclerosis |
|
Which nephrotic syndrome has selective proteinuria? (loss of albumin but not immunoglobulin) |
Minimal change disease |
|
Which nephrotic syndrome has an excellent response to steroids? |
Minimal change disease because the damage is mediated by cytokines from T cells and the steroids inhibit that. No other neprhotic syndromes respond to steroids. |
|
Most common cause of nephrotic syndrome in hispanics and african americans? |
Focal segmental glomerulosclerosis |
|
Which nephrotic syndrome is associated with HIV? |
Focal segmental glomerulosclerosis |
|
Which nephrotic syndrome is associated with heroin use? |
Focal segmental glomerulosclerosis |
|
Which nephrotic syndrome is associated with sickle cell disease? |
Focal segmental glomerulosclerosis |
|
What is focal segmental glomerulosclerosis? (break down the name) |
Focal = some glomeruli Segmental = involving only part of the glomerulus Sclerosis on H&E stain |
|
Which nephrotic syndrome has effacement of foot processes? (2) |
Minimal change disease and focal segmenting glomerulosclerosis |
|
Most common cause of nephrotic syndrome in caucasian adults |
Membranous nephropathy |
|
Which nephrotic syndrome is associated with hepatitis B or C? (2) |
Membranous nephropathy and Type 1 membranoproliferative glomerulonephritis |
|
Which nephrotic syndrome is associated with solid tumors? |
Membranous nephropathy |
|
Which nephrotic syndrome is associated with SLE? |
Membranous nephrophaty |
|
Which nephrotic syndrome is associated with drugs? |
Membranous nephropathy |
|
Which nephrotic syndrome has a "tram-track" appearance? |
Membranoproliferative glomerulonephritis |
|
Which nephrotic syndromes have immune complex deposition therefore have granular immunofluorescence? (2) |
Membranous nephropathy and membranoproliferative glomerulonephritis |
|
Which nephrotic syndrome has a "spike and dome" appearance on EM? |
Membranous nepropathy |
|
What are the two types of membranoproliferative glomerulonephritis? |
Type 1 = subendothelial Type 2 = dense deposit disease, intramembranous |
|
Which nephrotic syndrome is associated with an antibody called C3 nephritic factor? |
Type 2 Membranoproliferative glomerulonephritis |
|
Name the three nephrotic syndromes based on location: Subepithelial, intramembranous (basement membrane), subendothelial |
Subepithelial = membranous nephropathy Intramembranous (basement membrane) = Type 2 membranoproliferative glomerulonephritis Subendothelial = Type 1 membranoproliferative glomerulonephritis |
|
Which nephrotic syndrome can also cause nephritic syndrome? |
Membranoproliferative glomerulonephritis |
|
Why is GFR increased in diabetes induced nephrotic syndrome? |
The efferent arteriole is more affected than the afferent arteriole |
|
Why do ace inhibitors help the kidneys with patients who have diabetes mellitus? |
Angiotensin II constricts the efferent arteriole so by blocking it you block the vasoconstriction |
|
What are Kimmelstiel-Wilson nodules and what are they associated with? |
Sclerosis of mesangium of nephrons and is associated with diabetic nephropathy. Looks like an empty nephron filled with hyaline material. |
|
Which organ is most commonly involved in systemic amyloidosis? |
Kidney |
|
Which nephrotic syndrome is characterized by apple-green birefringence under polarized light? |
Nephrotic syndrome caused by systemic amyloidosis |
|
0-14 years old which leukemia? |
ALL |
|
15-39 which leukemia? |
AML blasts with auer rods |
|
40-59 which leukemia? |
AML and CML |
|
40-59 year old how do you differentiate AML from CML? |
AML has blasts greater than 30% |
|
60+ years old which leukemia? |
CLL |
|
4 hallmarks of nephritic syndrome that differentiates it from nephrotic syndrome |
Limited proteinuria <3.5g/day RBC casts and dysmorphic RBC in urine Glomerular inflammation Periorbital edema |
|
Nephritic syndrome that arises after group A B-hemolytic streptococcal infection of the skin (impetigo) or pharynx |
Poststreptococcal Glomerulonephritis (PSGN) |
|
Hallmark of this nephritic syndrome are subepithelial "humps" |
Poststreptococcal glomerulonephritis |
|
Nephritic syndrome that progresses to renal failure in weeks to months |
Rapidly progressive glomerulonephritis |
|
Which nephritic syndrome is characterized by crescents in bowmans space and what are those crescents comprised of? |
Rapidly progressive glomerulonephritis - crescents are made of fibrin and macrophages |
|
If IF pattern shows linear (anti-basement membrane antibody) which disease caused the rapidly progressive glomerluonephritis? |
Goodpasture syndrome |
|
If IF pattern show Granular(immune complex deposition) which disease caused the rapidly progressive glomerulonephritis? (2) |
PSGN or diffuse proliferative glomerulonephritis |
|
Rapidly progressive glomerulonephritis that is due to diffuse antigen-antibody complex deposition, usually sub-endothelial |
Diffuse proliferative glomerulonephritis |
|
Most common type of renal disease in SLE patients? |
Diffuse proliferative glomerulonephritis |
|
If IF pattern show negative IF (pauci-immune) which disease caused the rapidly progressive glomerulonephritis? (3) |
Wegener granulomatosis, microscopic polyangiitis, and Churg-Strauss sydnrome |
|
What distinguishes Churg-Strauss from microscopic polyangiitis? (3) |
Churg-Strauss has granulomatous inflammation, eosinophilia, and asthma. |
|
Which nephritic syndrome has IgA immune complex deposition in mesangium of glomeruli |
IgA Nephropathy (Berger Disease) |
|
Which nephritic syndrome has inherited defect in type IV collagen? |
Alport syndrome |
|
Alport syndrome presents with what three key findings? |
Isolated hematuria hearing loss ocular disturbances |
|
Infection of bladder |
Cystitis |
|
Gold standard measurement for Cystitis |
Culture - greater than 100,000 CFU |
|
Most common cause for cystitis (bacteria)? |
E. Coli 80% |
|
Cystitis with an alkaline urine and an ammonia scent is most likely due to which bacteria? |
Proteius mirabilis |
|
Name the 5 bacterial causes for cystitis with a positive culture? |
E. Coli Staph Saprophyticus Klebsiella pneumonia Proteus mirabilis Enetercoccus faecalis |
|
Sterile pyuria with a negative urine culture suggests infection with what? (2) |
Chlamydia trachomatis Neisseria gonorrhoeae |
|
Infection of kidney |
Pyelonephritis |
|
3 most common pathogens in pyelonephritis |
E. Coli Enterococcus faecalis Klebsiella species |
|
Interstitial fibrosis and atrophy of tubules due to multiple bouts of acute pyelonephritis |
Chronic pyelonephritis |
|
Atrophic tubules containing eosinophilic proteinaceous material resemble thyroid follicles suggests what? |
Chronic pyelonephritis |
|
Precipitation of a urinary solute as a stone |
Nephrolithiasis |
|
Treatment for calcium oxalate and/or calcium phosphate stones? |
Hyrdocholothiazide (calcium-sparing diuretic) - decreases amount of calcium going into urine. |
|
Most common cause of this nephrolith is idiopathic hypercalciuria |
Calcium oxalate and/or calcium phosphate |
|
List the four most common nephrolith in order from most to least. |
Calcium oxalate and/or calcium phosphate Ammonium magnesium phosphate Uric Acid Cysteine |
|
Most common cause of this nephrolith is infection with urease-positive organisms (proteius vulgaris or klebsiella). |
Ammonium magnesium phosphate |
|
What is the pathophysiology of the ammonium magnesium phosphate stone? |
Infection with a urease positive organism leads to alkaline urine which forms a stone. |
|
Which nephrolith classically results in a staghorn calculi in renal calyces? (2) one is for adults and one is for children |
Ammonium magnesium phosphate (adults) Cysteine (children) |
|
Which of the nephroliths are radiolucent? |
Uric acid |
|
This nephrolith is commonly seen in children |
Cysteine |
|
Where is EPO created? |
renal peritubular interstitial cells |
|
Hamartoma comprised of blood vessels, smooth muscle, and adipose tissue? |
Angiomyolipoma |
|
Angiomyolipoma have increased frequency in what? |
Tuberous sclerosis |
|
Malignant epithelial tumor arising from kidney tubules |
Renal Cell Carcinoma |
|
What is the classic triad for renal cell carcinoma |
Hematuria Palpable Mass Flank pain |
|
Which of the renal diseases can release excess EPO, renin, PTHrp, or ACTH? |
Renal cell carcinona - paraneoplastic syndrome |
|
Why do you only see left sided varicocele in renal cell carcinoma? why not right? |
Left spermatic vein drains into renal vein while right spermatic vein drains directly into IVC |
|
What kind of cells are seen in renal cell carcinoma? |
Clear cells |
|
What is the pathogenesis of renal cell carcinoma starting with loss of which gene? |
Loss of VHL (3p) tumor suppressor gene -increased IGF-1 promotes growth -increased HIF transcription factor (increases VEGF and PDGF) |
|
What is the key risk factor in the spontaneous pathway of renal cell carcinoma? |
Smoking |
|
What disease has an increased risk for renal cell carcinoma? and what other tumor does it have a increased risk for? |
Von Hippel-Lindau disease and hemangioblastoma of the cerebullum |
|
What is the inheritance pattern of Von Hippel-Lindau disease? |
Autosomal dominant |
|
Most common malignant renal tumor in children? |
Wilms tumor |
|
Malignant kidney tumor comprised of blastema, primitive glomeruli and tubules, and stromal cells |
Wilms tumor |
|
Wilms tumor is associated with what mutation? |
WT1 |
|
What does WAGR syndrome stand for? |
Wilms tumor Aniridia Genital Abnormalities Retardation |
|
What are the 4 features of Beckwith-Wiedemann syndrome? |
Wilms tumor Neonatal hypoglycemia Muscular hemihypertrophy Organomegaly especially tongue |
|
Malignant tumor arising from urothelial lining of renal pelvis, ureter, bladder, or urethra. |
Urothelial carcionma |
|
Azo dyes increased the risk for what cancer? |
Urothelial carcinoma |
|
Longterm cyclophosphamide or phenacetin can increase risk for what cancer? |
Urothelial carcinoma |
|
What are the two pathways that urothelial carcinoma arises? |
Flat and papillary |
|
|