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1623 Cards in this Set

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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