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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

Which pathway for urothelial carcioma is associated with early p53 mutations?

Flat pathway NOT papillary

Which pathway for urothelial carcinoma starts as a high grade and which starts as a low grade?

Flat: High grade --> invasivie


Papillary: Low grade --> high grade --> invasive

Chronic cystitis has an increased risk for what cancer?

Squamous cell carcinoma of lower urinary tract

Schitosoma hematobium infection has an increased risk for what cancer? (Egyptian male)

Squamous cell carcinoma of lower urinary tract

Long-standing nephrolithiasis has an increased risk for what cancer?

Squomous cell carcinoma of lower urinary tract

A urachal remnant has an increased risk for what cancer? what is the urachas? where does this cancer normally arise?

Adenocarcinoma of the bladder


Urachas - duct that connects the fetal bladder to the yolk sac, goes through the umbilical cord.


Arises in the dome of the bladder

Mannitol MOA

Osmotic diuretic

Acetazolamide MOA

Carbonic anhydrase inhibitor

What are the 2 loop diuretics? and what is the MOA?

Furosemide and Ethacrynic acid


Inhibits cotransport system (Na/K/2CL). Prevents concentration of urine.

MOA of hydrochlorothiazide

Inhibits NaCL reabsorption

Name the 4 K+ sparing diuretics

Spironolactone


Triamterene


Amiloride


Epelernone

MOA of triamterene and amiloride

Blocks Na channels in the cortical collecting tubules

MOA of spironolactone and eplerenone

Aldosterone receptor antagonists

Where is the bartholin gland located and what is it's purpose?

Located in the vaginal canal and produces mucus-like fluid

Cystic dilation of the bartholin gland due to an obstruction

Bartholin cyst

Warty neoplasm of vulvar skin

Condyloma

Which viruses cause condyloma?

HPV 6 or 11

Histologically what are HPV-assocaited condylomas characterized by? and what does it look like?

koilocytes - looks like a raisin

What are the low risk HPVs and what are the high risk HPVs?

low risk = 6 and 11


high risk = 16,18,31,33

This vulvar lesion is characterized by thinning of the epidermis and fibrosis of the dermis

Lichen sclerosis

Parchment-like vulvar skin is indicative of what pathology?

Lichen sclerosis

This vulvar pathology is characterized by hyperplasia of vulvar squamous epithelium

Lichen simplex chronicus

Carcinoma arising from squamous epithelium lining the vulva

Vulvar carcinoma

Two types of vulvar carcinoma and what are their causes

HPV-related = HPV 16 and 18


Non-HPV-related = long standing lichen sclerosis

Malignant epithelial cell in the epidermis of the vulva

Extramammary paget disease

Paget cells vs Melanoma stains: PAS, Keratin, S100

Paget = PAS+, Keratin+


Melanoma = S-100+

Major side effect of amphotericin B (2)

Hypokalemia, hypomagnesia

Difference between paget disease of vulva vs nipple regarding underlying carcinoma

Vulva = no underlying carcinoma


Nipple = underlying carcinoma

Focal persistence of columnar epithelium in the upper vagina as opposed to squamous epithelium

Adenosis

Adenosis has increased incidence in females exposed to what?

DES (diethylstilbestrol) estrogen like

Malignant proliferation of glands with clear cytoplasm in the vagina

Clear cell adenocarcinoma

Feared complication of DES-adenosis of the vagina

Clear cell adenocarcinoma

DES (diethylstilbestrol) moms have a slight increased risk for what?

Breast carcinoma

Malignant mesenchymal proliferation of immature skeletal muscle

Embryonal rhabdomyosarcoma

This pathology presents as bleeding and grape-like mass protruding from vagina or penis of child

Embryonal rhabdomyosarcoma

Rhabdomyoblast will have positive IHC staining for what 2 things?

Desmin and myogenin

Carcinoma arising from squamous epithelium lining the vaginal mucosa

Vaginal carcinoma

Cancer from the lower 1/3 of the vagina goes to which lymph nodes? upper 2/3 of the vagina?

lower 1/3 = inguinal nodes


upper 2/3 = iliac nodes

What is the exocervix and endocervix lined by?

Exocervix = squamous epithelium


Endocervix = columnar epithelium

What makes high-risk HPV high risk?

EG: increases destruction of p53


E7: increases destruction of Rb

4 grades of cervical intraepithelial neoplasia and what defines each

CIN 1: involves 1/3 thickness of epithelium


CIN 2: involves 2/3


CIN 3: slightly less than full thickness


Carcinoma In Situ = Full thickness

Invasive carcinoma that arises from cervical epithelium

Cervical carcinoma

2 secondary risk factors for in HPV related cervical carcinoma

Smoking and immunodeficiency

Two subtypes of cervical carcinoma

squamous cell carcinoma and adenocarcinoma

Confirmatory test for an abnormal pap smear

Colposcopy and biopsy

Pap smear does not screen which carcinoma very well?

adenocarcinoma

Formula for fractional filtration

FF = GFR/RPF

Maximal effect a drug can produce is known as?

Efficacy

Amount of drug needed for a given effect is known as?

Potency

Which interleukin is an endogenous pyrogen and also is osteoclast-activating?

IL-1

What hormones are responsible for each of the phases of menstrual cycle. Proliferative, secretory, menstrual

Proliferative = estrogen


Secretory = progesterone


Menstrual = loss of progesterone

Secondary amenorrhea due to loss of basalis (regenerative layer) and scarring of the endometrium

Asherman Syndrome - due to overaggressive dilation and currettage

Lack of ovulation is known as?

Anovulatory cycle

Anovulatory cycle can cause what (pathophysiology)?

Estrogen-driven proliferative phase without progesterone secretory phase. Leads to glands being laid on top of each other. This can cause dysfunctional uterine bleeding.

Bacterial infection of endometrium

Acute endometritis

Chronic inflammation of the endometrium

Chronic endometritis

Lymphocytes are normally seen in the endometrium, what cell must be present in order for it to be chronic endometritis?

Plasma cells

Hyperplastic protusion of endometrium

Endometrial polyp

Endometrial polyp can be a side-effect of what drug?

Tamoxifen

Endometrial glands and stroma outside of the uterine endometrial lining

Endometriosis

Formation of a chocolate cyst in the ovaries is suggestive of what disease?

Endometriosis

Endometriosis with involvement of the uterine myometrium is called?

adenomyosis

Hyperplasia of endometrial glands relative to stroma

Endometrial hyperplasia

What is a consequence of unopposed estrogen?

Endometrial hyperplasia

Endometrial hyperplasia: histologically what is the most important predictor for progression to carcinoma?

Cellular atypia

Malignant proliferation of endometrial glands

Endometrial carcinoma

What are the two pathways for endometrial carcinoma and which is most common?

Hyperplasia pathway (75%)


Sporadic pathway (25%)

What is the histology of hyperplasia and sporadic pathways of endometrial carcinoma? (2)

hyperplasia = endometriod (normal endometrium like)


sporadic = papillary serous

Age differences for hyperplasia vs sporadic pathways of endometrial carcinoma?

hyperplasia = 50 years old


sporadic = 70 years old

Sporadic pathway of endometrial carcinoma is driven by what mutation?

p53

What are psammoma bodies and what is it associated with? (female GS)

Concentric calcifications - Endometrial carcinoma (sporadic pathway) (serious papillary)

What diseases are psammoma bodies found? (4)

Papillary carcinoma of the thyroid


Meningioma


Papillary serous endometrial carcinoma


Mesothelioma


Benign proliferation of smooth muscle arising from myometrium.

Leiomyoma

Describe a leiomyoma

Multiple, well-defined white whorled masses

Are Leiomyomas usually symptomatic or asymptomatic?

asymptomatic

Malignant proliferation of smooth muscle arising from the myometrium

Leiomyosarcoma

What is the precursor to leiomyosarcoma?

It arises de novo, it does NOT arise from leiomyoma

Which has multiple and which has a single lesion? Leiomyoma and leiomyosarcoma

Leiomyoma = multiple


Leiomyosarcoma = single

What hormones affects theca cells? and what does it produce because of it?

Leutenizing hormone allows theca cells to make androgen

what hormone affects granulosa cells? and does it produce because of it?

FSH - which allows the conversion of androgen from theca cells to estradiol for the egg

What does a residual follicle become? (Female GS)

Corpeus luteum

What does the corpus luteum secrete and what is it's main job?

Secretes progesterone which prepares the endometrium for maintenance and possible pregnancy

What hormone cascasde causes ovulation?

Estradiol surge induces a LH surge

Multiple ovarian follicular cysts due to hormone imbalance

Polycystic ovarian disease

What hormone is elevated and what hormone is decreased in polycystic ovarian disease?

LH = increased


FSH = low

What are the three cell types of the ovary that tumors can arise from?

Surface epithelial, germ cells, and sex-cord stroma

What are the four subtypes of surface epithelial tumors of the ovaries?

Mucinous


Serous


Endometrioid


Brenner

Of the four subtypes of surface epithelial tumors which ones are cystic?

Serous and mucinous

Benign tumors of mucinous or serous origin of the ovaries are known as?

Cystadenomas

What age do cystadenomas normally present? cystadenocarcinoma? (Female GI)

cystadenoma: 35 years old (premenopausal)


cystadenocarcinoma: 65 years old (postmenopausal)

Difference in gross presentation between a cystadenoma vs cystadenocarcinoma?

benign = single cysts with simple, flat linning


malignant = complex cysts with thick, shaggy lining


Malignant tumors of mucinous or serous origin of the ovaries are known as?

Cystadenocarcinoma

BRCA 1 carries have increased risk for what cancers?

Serous carcinoma of ovary


Serous carcinoma of fallopian tube


Breast cancer

Surface epithelial tumors of the ovaries that are composed of endometrial-like glands

Endometrioid tumors

Which surface epithelial tumor of the ovary is associated with endometriosis

Endometrioid tumor

Surface epithelial tumor of the ovary that contains urothelium

Brenner tumor

Usually malignant or benign? Endometrioid tumor, Brenner tumor? (female GI)

Endometrioid usually malignant


Brenner usually benign

Marker for surface epithelial tumor of ovaries

CA-125

Five subtypes of germ cell ovarian tumors (use tissues that are produced for help)

Fetal tissue- Cystic teratoma


Fetal tissue - Embryonal carcioma


Oocytes - Dysgerminoma


Yolk sac - Endodermal Sinus tumor


Placental tissue - Choriocarcinoma

Cystic tumor in the ovary that is derived from two or three embryologic layers

Cystic teratoma

What is the most common type of tissue that is immature in an immature teratoma?

Neuroectoderm

Cystic teratoma composed primarily of thyroid tissue

Struma ovarii

Germ cell tumor of the ovary that is composoed of large cells with clear cytoplasm and central nuclei

Dysgerminoma

Testicular counterpart to dysgerminoma

Seminoma

Malignant germ cell tumor of the ovary that mimics the yolk sac

Endodermal sinus tumor

Most common ovarian germ cell tumor in children

Endodermal sinus tumor (yolk sac)

Which ovarian germ cell tumor has increased alpha fetal protein?

Endodermal sinus tumor

What are schiller-duval bodies and what ovarian germ cell tumor is it classical seen in?

Glomeruloid like structures - Endodermal sinus tumor pg.146

Malignant proliferation of trophoblast and synctiotrophoblast

Choriocarcinoma

This germ cell tumor of the ovary has high beta-hCG

Choriocarcinoma

Which of the germ cell tumors have a poor response to chemotherapy?

Choriocarcinoma

Malignant germ cell tumor composed of large primitive cells

Embryonal carcinoma

Neoplasm of granulosa and theca cells

Granulosa-theca cell tumor

Granulosa-theca cell tumors often produce what hormone?

Estrogen

Which sex cord-stromal tumor of the ovary has Reinke crystals

Sertoli-Leydig cell tumor

Which sex cord-stromal tumor of the ovary produces androgen?

Sertoli-Leydig cell tumor

A woman with pleural effusion and ascites as well as a large mass in the ovary has what syndrome? and what is that mass?

Meig's syndrome - fibroma

Pseudomyxoma peritonei is massive amounts of mucus in the periotenum where does the tumor come from?

Mucinous carcinoma of the appendix

Pseudomyxoma peritonei is usually caused by mucinous carcinoma of the appendix where is the most common metastasis site?

Ovaries

Implantation of fertilized ovum at site other than uterine wall

Ectopic pregnancy

Lower quadrant abdominal pain weeks after missed period suggests what?

Ectopic pregnancy

Spontaneous abortions are most often due to what?

Chromosomal anomalies especially chromosome 16

Teratogenic effects of alcohol (3)

Mental retardation


Facial abnormalities


Microcephaly

Teratogenic effects of cocaine (2)

intrauterine growth retardation


placental abruption

Teratogenic effects of Thalidomide

Limb defects

Teratogenic effects of cigarette smoke

Intrauterine growth retardation

Teratogenic effects of isotretinoin (3)

Spontaneous abortion


Hearing impairment


Visual impairment

Teratogenic effects of warfarin

Fetal bleeding

Teratogenic effects of phenytoin

Digit hypoplasia


Cleft lip/palate

Implantation of placenta in lower uterine segment

Placenta previa (preview of the placenta through the cervical os (opening))

Separation of placenta from uterine wall (decidua) prior to delivery of fetus leading to still birth

Placental abruption

Improper implantation of placenta into myometrium with little or no intervening decidua

Placenta accreta

Pregnancy induced HTN, proteinuria, and edema

Preeclampsia

What is HELLP and what is it used for?

Preeclampsia


Hemolysis


Elevated Liver enzyemes


Low Platelets

What is preeclampsia due to?

Abnormality of maternal-fetal vascular interface

What is pre-eclampsia with seizures?

Eclampsia

Abnormal conception characterized by swollen and edematous villi with proliferation of trophoblasts

Hydatidiform mole

This classically presents in the second trimester as passage of grape-like masses through vaginal canal

Hydatidiform mole

Feared complication of hydatidiform mole?

Choriocarcinoma

Two ways chriocarcinoma can arise? which responds well to chemotherapy? which does not?

Gestation pathway - responds well


Germ cell pathway - does not respond well

Opening of urethra on inferior surface of penis

Hypospadias

What is hypospadias due to?

Failure of the urethral folds to close

Opening of urethra on superior surface of penis

Epispadias

What is epispadias due to?

Due to abnormal positioning of the genital tubercle

Necrotizing granulomatous inflammation of the inguinal lymphatics and lymph nodes

Lymphogranuloma Venereum

Benign warty growth on genital skin

Condyloma Acuminatum

Condyloma Acuminatum is due to what?

HPV 6 or 11

Chlamydia trachomatis (L1-L3) can cause what STD which involves the lymph nodes?

Lymphogranuloma Venereum

Malignant proliferation of squamous cell of penile skin

Squamous cell carcinoma

In situ carcinoma of the penile shaft or scortum that presents as leukoplakia

Bowen disease

In situ carcinoma on the glans that presents as erythroplakia

Erythroplasia of Queyrat

In situ carcinoma that presents as multiple reddish papules on the penis

Bowenoid papulosis

Failure of testicle to descend into scrotal

Crytpoorchidism

Inflammation of testicle

Orchitis

Orchitis seen in young adults is normally caused by what two organisms?

Chlamydia trachomatis (D-K)


Neisseria Gonorrhoeae

Orchitis seen in older adults is normally caused by what two organisms?

E. Coli


Pseudomonas

Orchitis seen in teenage males is normally caused by what organism?

Mumps virus

This is characterized by granulomas involving seminiferous tubules

Autoimmune orchitis

Twisting of the spermatic cord leading to congestion and hemorrhagic infarction

Testicular torsion

Granulomas in the testicle can be caused by what two things?

TB -necrotizing granulomas


autoimmune orchitis - granulomas

Absent cremasteric reflex (touching inner thigh leading to scrotum to move up) is indicative of what?

Testicular torsion

Dilation of spermatic vein due to impaired drainage

Varicocele

Presents as a scrotal swelling with "bag of worms' appearance

Varicocele

Varicocele are usually on what side?

Left side

Fluid collection within tunica vaginalis of the testicle

Hydrocele

What are the two types of testicular tumors?

Germ cells or sex-cord stroma

Why are testicular tumors not biopsied?

Risk of seeding scrotum; removed via radical orchiectomy

Two risk factors for germ cell tumors in males

Cryptorchidism and Klinefelter syndrome

What are the two types of germ cell tumors of the testicles?

Seminoma and nonseminoma

Which of the two subtypes of germ cell tumors of the testicles are highly responsive to radiotherapy?

Seminoma not nonseminoma

Which of the two subtypes of germ cell tumors of the testicles metastasizes late?

Seminoma not nonseminoma

Which of the two subtypes of germ cell tumors of the testicles has an excellent prognosis?

Seminoma not nonseminomas

Malignant tumor of the testicle with large cells with clear cytoplasm and central nuclei

Seminoma, similar description to dysgerminoma

Which of the testicular tumors is a homogenous mass with no hemorrhage or necrosis?

Seminoma

Which testicular cancer can produce beta-hCG although it is rare to happen?

Seminoma

What are the 4 nonseminomas of the testicular?

Embryonal carcinoma


Yolk sac (endodermal sinus) tumor


Choriocarcinoma


Teratorma

Malignant tumor of testicles made of immature, primitive cells that may form glands.

Embryonal carcinoma

This testicular tumor is a hemorrhage mass with necrosis

Embryonal carcinoma

Chemotherapy may cause this testicular tumor to differntiate

Embryonal carcinoma

These testicular tumors has increased AFP or beta-hCG (2)

Embryonal carcinoma and Teratoma

Testicular tumor that resembles a yolk sac

Yolk sac tumor (endodermal sinus)

Most common testicular tumor in children

Yolk sac tumor

AFP is elevated in this testicular tumor

Yolk sac tumor

Schiller-Duval bodies are found in which testicular tumor?

Yolk sac tumor (endodermal sinus)

Malignant testicular tumor of synctiotrophoblasts and cytotrophoblasts

Choriocarcinoma

B-hCG is elevated in this testicular carcinoma

Choriocarcinoma

Teratoma is malignant in which sex?

Males

Lymphoma of testicles are usually composed of what?

Diffuse large B-cell type

Leydig cell tumors produce what hormone?

Androgen

Which sex-cord stromal tumor is comprised of tubules?

Sertoli cell tumor

Acute inflammation of prostate, usually due to bacteria

Acute prostatitis

Which two organisms are responsible for acute prostatitis in young adults?

C trachomatis


N gonorrhoeae

Which two organisms are responsible for Acute prostatitis in older adults?

E Coli


Pseudomonas

Chronic inflammation of prostate

Chronic prostatitis

Hyperplasia of prostatic stroma and glands

Benign Prostatic Hyperplasia (BPH)

Where does BPH occur within the prostate?

Periurethral zone

Conversion of what hormone to what other hormone causes BPH? what enzyme is responsible for the conversion?

Testosterone --5a-reductase--> DHT

What two types of drugs are used for BPH?

A1 antagonists and 5a-reductase inhibitors

Alpha inhibitors can be used to treat BPH what are the two types and what are the indications for each?

A1-antagonist (relax smooth muscles) - terazosin


Selective A1a-antagonist so that it does not effect blood vessels in normotensive individuals - tamulosin

Malignant proliferation of prostatic glands

Prostate adenocarcinoma

What grading system is used for prostate adenocarcinoma? This grading system is not based on atypia but instead base on what?

Gleason grading system - based on architecture

MOA of leuprolide and what is it used for?

Continuous - GnRH analog that shut does the anterior pituitary gonadotrophs (LH and FSH). used for prostate adenocarcinoma


Pulsatile - agonist when used pulsatile

MOA of flutamide and what is it used for?

Antagonist of androgen receptor used for prostate adenocarcinoma

Common metastasis site for prostate adenocarcinoma

lumbar spine

MOA of terazosin and what is it used for?

A1 antagonist. HTN and BPH

MOA of tamulosin and what is it used for?

Selective A1a antagonist. BPH in normotensive patients

Benign rathke pouch tumor with cholesterol crystals, calcifications

Craniopharyngioma

What is the role of the synctiotrophoblast and which layer?

Secrete hCG (similar to LH), which stimulates the corpeus luteum to continue secreting progesterone. Outer layer

What is the role of the cytotrophoblast? which layer?

Inner layer which makes cells

Umbilical artery contains oxygenated or deoxygenated blood?

Deoxygenated

MOA of finasteride

Inhibits 5a-reductase leading to decreased formation of DHT from testosterone

Chromosomes of Klinefelter

XXY

Chromosomes of Turner syndrome

XO

Which hydatidiform mole has fetal parts and which does not have fetal parts?

Partial = partial fetus


Complete = No fetal parts

MOA of Clomiphene and what is it used for?

Antagonist at estrogen receptor in hypthalamus which blocks feedback inhibition and increase LH and FSH. Used for infertility.

MOA of Tamoxifen

Estrogen antagonist at breast tissue.

MOA of Raloxifene and what is it used for?

Estrogen agonist on bone. Osteoporosis

MOA of Mifepristone (RU-486) and what is it used for?

Competitive inhibitor of progestins at progesterone receptors. Used to terminate pregnancy. Used with misoprostol (PGE1)

MOA of Terbutaline and what is it used for?

B2 - agonist that relaxes the uterus, used to decrease contraction frequency of women during labor

MOA of Danazol and what is it used for?

Synthetic androgen, partial agonist at androgen receptors. Used for endometriosis and hereditary angioedema.

MOA of flutamide and what is it used for?

Nonsteroidal inhibitor of androgens at the testosterone receptor. Used in prostate carcinoma.

MOA of ketoconazole and what is it used for?

Inhibit steroid synthesis (inhibits 17,20-desmolase). Used for PCOD to prevent hirsutism.

MOA of spironolactone and what is it used for?

Inhibit steroid binding (17a-hydroxylase, 17-20 desmolase). Used for PCOD to prevent hirsutism.



K+ sparing diuretic - aldosterone receptor antagonist

MOA of sildenafil and what is it used for?

Inhibit phosphodiesterase 5, leading to increase of cGMP. Erectile dysfunction.

MOA of vardenafil and what is it used for?

Inhibit phosphodiesterase 5, leading to increase in cGMP. Erectile dysfunction.

What does adnexal mass refer to?

A lump of tissue in the adnexa of the uterus

What prevents lactation during pregnancy even though prolactin is elevated?

Estrogen and progesterone

A 32 year old female presents to your office complaining of small amount of vaginal discharge. Application of KOH solution to the discharge yields a strong fishy odor. What is the dx? and organism responsible?

Bacterial vaginosis caused most commonly by Gardnerella vaginalis

Primary amennorrhea in a pt with fully developed secondary sexual characteristics is most commonly due to what two things?

Imperforate hymen or mullerian duct anomaly

Imperfrorate hymen presents with what three key characteristics?

Primary amennorrhea


Cyclical pain


Palpable mass anterior to the rectum

What hormone causes maternal insulin resistance?

human placental lactogen

Define primigravada

A woman who is pregnant for the first time

What vitamins are not found in breast milk?

Vitamin D and K

Attrition bias is what type of bias? Bias that occurs because people die in a study

Selection bias

Which cells in the testicle produce testosterone?

Leydig cells in response to LH

Which cells nurse the sperm?

Sertoli cells

Nonfunctional tumors of the pituitary present with mass effect: what are the 3 symptoms?

Bitemporal hemianopsia


Hypopituitarism


Headache

Most common pituitary tumor?

Prolactinoma

How does a prolactinoma cause amenorrhea?

Prolactin inhibits GnRH which decreases LH and FSH leading to amenorrhea

Galactorrhea and amenorrhea suggests what tumor?

Prolactinoma

Decreased libido and headaches in a male suggests what type of endocrine tumor?

Prolactinoma

How do you treat prolactinoma? (2)

dopamine agonists or surgery

MOA of bromocriptine and what is it used for (2)?

Dopamine agonist used for prolactinomas and for parkinson's

MOA of cabergoline and what is it used for?

Dopamine agonist used for prolactinomas

How does a growth hormone adenoma cause secondary diabetes mellitus?

GH induces liver gluconeogenesis

In growth hormone adenoma Growth hormone is not responsible fro the growth of tissue, instead it activates something else. What is that?

IGF-1

MOA of octreotide

Somatstatin analog, blocks release of GH.

Pregnancy-related infarction of the pituitary gland

Sheehan syndrome

Pathophysiology of Sheehan syndrome

Gland doubles in size during pregnancy, but blood supply does not increase significantly; blood loss during parturition precipitates infarction.

A pt after giving birth presents with poor lactation, loss of pubic hair, and fatigue. What is the dx?

Sheehan syndrome

Herniation of the arachnoid and CF into the sella compresses and destroys the pituitary gland

Empty sella syndrome

What hormones are produced in the posterior pituitary and how is it transported?

ADH and oxytocin which are transported via axons from hypothalamus


ADH deficiency leads to what?

Centra diabetes insipidus

Impaired renal response to ADH

Nephrogenic diabetes insipidus

MOA of desmopressin and what is it used for?

ADH analog used for central diabetes insipidus

Excessive ADH secretion

Syndrome of inappropriate ADH (SIADH) secretion

What is the treatment for syndrome of inappropriate ADH secretion

demeclocycline - blocks the affect of ADH

A patient who has syndrome of inappropriate ADH secretion and cancer most likely has what kind of cancer?

Small cell carcinoma of the lung

How does hyperthyroidism increase BMR?

increased synthesis of Na-K ATPase

How does hyperthyroidism increase sympathetic nervous system activity?

Increased expression of B1-adrenergic receptors

Hyperthyroidism has increased or decreased cholesterol and glucose levels?

Hypocholesterolemia


Hyperglycemia

Autoantibody that stimulate TSH receptor

Graves disease

What type of antibody is responsible for Grave's disease?

IgG

What type of hypersensitivity is grave's disease?

Type II

What is exophthalmos due to in grave's disease?

Fibroblasts behind the orbit express the TSH receptor. Not the increased thyroid hormone.

Scalloped colloid is found in what endocrine disorder?

Grave's disease

MOA of PTU (propylthiouracil)

Inhibits peroxidase-mediated oxidation, organification, and coupling steps of thyroid synthesis as well as peripheral conversion of T4 to T3.

Hypothyroidism in neonates and infants

Cretinism

Most common enzyme to be deficient in cretinism?

thyroid peroxidase

Hypothyroidism in an older child or adult

Myxedma

Autoimmune destruction of thyroid gland

Hashimoto Thyroiditis

Hashimoto thyroiditis is associated with what HLA?

HLA-DR5

What are hurthle cells and what are they associated with?

Hurthle cells are eosinophilic metaplasia of cells that line follices. Associated with hashimoto's thyroiditis

Which thyroid disorder has chronic inflammation with germinal centers?

Hashimoto Thyroiditis

Hashimoto thyroiditis has an increased for what kind of lymphoma?

B-cell (marginal zone) lymphoma

A pt presents with hashimoto thyroiditis and an enlarging thyroid gland what is most likely the cause?

B-cell (marginal zone) lymphoma

Granulomatous thyroiditis that follows a viral infection

Subacute (deQuervain) Granulomatous thyroiditis

A young woman presents with tender thyroid what is the most likely dx?

Subacute (deQuervain) granulomatous thyroiditis

Chronic inflammation with extensive fibrosis of thyroid

Reidel fibrosing thyroiditis

Reidel fibrosing thyroiditis hyper or hypo thyroidism?

Hypothyroidism

Subacute (de quervain) granulomatous thyroiditis hyper or hypo thyroidism?

Hyperthyroidism may progress to hypothyroidism

A patient presents with a hard as wood nontender thyroid gland?

Reidel fibrosing thyroiditis

A YOUNG patient with a fibrosing thyroid that is invading local structures like the airway is most likely what?

Reidel fibrosing thyroiditis since they are young, if they were older then more likely anaplastic carcinoma.

How do you biopsy the thyroid? and why is that technique used?

Fine needle aspiration (FNA) because the thyroid is very vascular

Benign proliferation of follicles surrounded by a fibrous capsule (thyroid)

Follicular adenoma

Which thyroid neoplasia has a fibrous capsule?

Follicular adenoma

What are the four types of thyroid carcinomas?

Papillary


Follicular


Medullary


Anaplastic

Which thyroid carcinoma's major risk factor is ionizing radiation in childhood?

Papillary carcinoma

What is Orphan Annie eye nuclei and what are they associated with?

Orphan annie eye nuclei are nuclei with a central white clearing. It is associated with papillary carcinoma.

Which thyroid carcinoma has psammoma bodies?

Papillary carcinoma

Which nodes do papillary carcinomas usually spread to?

Cervical nodes

Malignant proliferation of follicles (thyroid)

Follicular carcinoma

Which thyroid neoplasia has a fibrous capsule with invasion through capsule?

Follicular carcinoma

Why can you not differentiate follicular adenoma from follicular carcinoma with fine needle aspiration?

Follicular adenoma and follicular carcinoma histologically look the same, the difference is in the capsule integrity which can not be seen with FNA. Follicular adneoma has an intact capsule, follicular carcinoma does not.

Which four carcinomas like to spread via blood?

Renal cell carcinoma - renal viens


Follicular carcinoma


Hepatocellular carcinoma - hepatic veins


Choriocarcinoma


Malignant proliferation of parafollicular C cells

Medullary carcinoma

Which thyroid carcinoma has high levels of calcitonin?

Medullary carcinoma

Fine need aspiration that shows malignant cells in an amyloid stroma is indicative of what thyroid neoplasia?

Medullary carcinoma

Detection of what mutation warrants prophylactic thyroidectomy and why?

RET mutation, thyroid may progress to medullary carcinoma

Undifferentiated malignant tumor of thyroid

Anaplastic carcinoma

Which thyroid neoplasia likes to invade local structures?

Anaplastic carcinoma

What are the three actions of PTH?

Increase bone osteoclast activity


Increase small bowel absorption of calcium and phosphate


Increase renal calcium reabsorption


Decrease renal phosphate reabsorption

Excess PTH due to disorder of the parathyroid gland

Primary Hyperparathyroidism

Pituitary adenoma can cause what to the pancreas?

Acute pancreatitis

Osteitis fibrosa cystica is associated with what endocrine disorder?

Parathyroid adenoma

Nephrocalcinosis is associated with what endocrine disorder?

Parathyroid adenoma

Lab findings for parathyroid adenoma: increased or decreased for PTH, calcium, phsophate, cAMP, serum alkaline phosphatase

PTH increased


Calcium Increased


Phosphate decreased


Urinary cAMP increased


Serum alkaline phosphatase increased

Excess production of PTH due to a disease process extrinsic to the parathyroid gland

Secondary hyperparathyroidism

Most common cause for secondary hypoparathyroidism

Chronic renal failure

How does chronic renal failure cause secondary hyperparathyroidism?

Renal insufficiency leads to decreased phosphate excretion


Increased serum phosphate binds calcium


Low free calcium stimulate parathyroids


Increased PTH leads to bone resorption

Lab findings for secondary hypoparathyroidism due to CRF: Increased or decreased PTH, serum calcium, serum phosphate, alkaline phosphtase

PTH increased


Serum calcium decreased


Serum phosphate increased


Alkaline phosphatase increased

What endocrine disorder can occur due to DiGeorge syndrome?

Hypoparathyroidism

What is trousseau sign and what does it indicate?

tetany that is elicited with filling of blood pressure cuff - hypoparathyroidism

What is Chvostek sign and what does it indicate?

Tapping of facial nerve leads to spams - hypoparaythyroidism

End-organ resistance to PTH is called

Pseudohypoparathyroidism

What protein is defective in pseudohypoparathyroidism?

Gs (G stimulatory protein)

What are the lab values for calcium and PTH in pseudohypoparathyroidism?

Decreased calcium


Increased PTH

A pt with short stature and short 4th and 5th digits most likely has what endocrine disorder?

Pseudohypoparathyroidism

What cells secrete insulin and where are they relative to the islets?

Beta cells; lie in center of islets

What cells secrete glucagon?

Alpha cells

What cells destroys the beta cells in type 1 diabetes mellitus?

T Lymphocytes

Which HLA is type 1 diabetes associated with?

HLA-DR3 and DR4

What is the mechanism of insulin resistance in type II diabetes?

Obesity leads to decreased numbers of insulin receptors

Glucose can freely enter what areas without insulin?

Schwann cells


pericytes of retinal blood vessels


lens

What enzyme is responsible for converting glucose to sorbitol, resulting in osmotic damage in diabetes?

Aldose reductase

What are the three parts of MEN type 1?

3 P's


Pituitary Tumors


Parathyroid Tumors


Pancreatic Tumors

What are the three parts of MEN type 2A?

2P's


Parathyroid hyperplasia


Pheochromocytoma


Medullary thyroid carcinoma

What are the three parts of MEN type 2b?

1P


Pheochromocytoma


Medullary thyroid carcinoma


Mucosal neuromas

This pancreatic endocrine neoplasm presents with episodic hypoglycemia with mental status changes that are relieved by administration of glucose?

Insulinoma

This pancreatic endocrine neoplasm presents with treatment-resistant peptic ulcers

Gastrinomas (Zollinger-Ellison syndrome)

This pancreatic endocrine neoplasm presents with achlorhydria and cholelithiasis with steatorrhea

Somatostatinomas


achlorhydria - inhibits gastrin


steatorrhea - inhibits CCK

This pancreatic endocrine neoplasm presents with watery diarrhea, hypokalemia, and achlorhydria

VIPomas

Which pancreatic endocrine neoplasm has cholelithiasis?

Somatostatinoma

Adrenal cortex has three layers and what do each of the layers produce?

Glomerulosa - mineralocorticoids


Fasciculata - glucocoritcoids


Reticularis - sex steroids

Endocrine disorder with excess cortisol

Cushing syndrome

What are the three ways cortisol suppresses the immune system?

Inhibits these three things:


Phospholipase A2 = inflammatory mediator


IL-2 = T-cell growth factor


Histamines = vasodilation

What are the 4 causes for cushing syndrome?

Exogenous corticosteroids


Primary adrenal adenoma, hyperplasia or carcinoma


ACTH-secreting pituitary adenoma


Paraneoplastic ACTH secretion

How does a dexamethasone test distinguish pituitary vs small cell carcinoma cause for cushing?

Dexamethasone is a powerful cortisol analog which should suppress ACTH. In pituitary adenoma cortisol will be decreased due to negative feedback of ACTH. In small cell cell carcinoma cortisol remains high because there is no negative feedback.

Endocrine disorder with excess aldosterone

Conn syndrome

Excess aldosterone (conn syndrome) will cause what 3 things to happen in the collecting tubles of the kidney? what two cells are they affecting?


Principle cell:


Reabsorb Na --> Hypernatremia


Secrete K --> Hypokalemia


alpha-intercalated cell:


Secrete H+ --> metabolic alkalosis


In primary hyperaldosteronism what are the levels of aldosterone and renin?

Aldosterone is high because it is being secreted by an adenoma


Renin is low because high blood pressure lowers renin

In secondary hyperaldosteronism what are the levels of aldosterone and renin?

Renin is high because the renin-angiotensin system is activated


Aldosterone is high because Angiotensin II caused its release

Excess steroids with hyperplasia of both adrenal glands

Congential adrenal hyperplasia

What is deficient in congenital adrenal hyperplasia?

21-hydroxylase

What is the pathophysiology of congenital adrenal hyperplasia?

21-hydroxylase deficiency leads to increased production of sex steroids. Cholesterol is converted to pregnenolone which then converted to the 3 steroids (mineral, cortico, and sex). Mineral and cortico require 21 hydroxylase but sex does not so there is a lot of sex steroids. Deficiency of cortico leads to increase in ACTH which causes bilateral adrenal hyperplasia

If a patient presents with 21-hydroxylase deficiency symptoms but does not have salt wasting, hypokalemia, or hypovolemia what should you suspect and why?

11-hydroxylase deficiency because a little mineralocorticoids are still being produced by corticosteroids are still deficient.

What syndrome has adrenal insufficiency?

Waterhouse-Friderichsen syndrome

Pathophysiology of Waterhouse-Friederichsen syndrome?

N meningitidis infection leads to DIC which leads to hemorrhagic necrosis of adrenal glands

Sack of blood adrenals is associated with what?

Waterhous-Friederichsen syndrome

What are the 3 causes for chronic adrenal insufficiency?

Autoimmune destruction


TB


Metastatic carcinoma (e.g. lungs)

What is the adrenal medulla derived from?

Neural crest-derived chromaffin cells

Tumor of chromaffin cell

Pheochromoctyoma

A brown tumor of the adrenal gland suggests what pathology?

Pheochromocytoma

MOA of phenoxybenzamine and what is it used for?

Irreversible a-blocker used for pheochromocytoma

What is increased in serum in pheochromocytoma? (2)

metanephrines


vanillylmandelic acid

What are the rule of 10s for pheochromocytoma? (4)

10% bilateral


10% familial


10% malignant


10% located outside of adrenal medulla

Where do pheochromoctyomas like go if they are not in the adrenals?

bladder wall

What syndromes are associated with pheochromocytoma?

MEN 2A


MEN 2B


VHL disease


NF type 1

What are the Na, K, and plasma levels in congenital adrenal hyperplasia?

Decreased Na


Increased K


Hypvolemia

What is IL-4 responsible for?

IgE class switch

What is IL-10 responsible for?

inhibit Th1; stimulate Th2

What is IL-2 responsible for?

T-cell growth factor

What are the two layers of of epithelium in lobules and ducts of the breast? which one is outer and which is inner? what is the function of each?

Luminal = inner, makes milk


Myoepithelial = outer, contractile ejection of milk

What hormones cause the breast tissue to develop?

Estrogen and progesterone

Bacterial infection of the breast, usually S. aureus

Acute mastitis

Acute mastitis is usually due to what bacteria?

S. aureus

Inflammation of the subareolar ducts in breast

Periductal mastitis

Periductal mastitis has a relative deficiency in what vitamin?

Vitamin A

This breast infection presents with nipple retraction, but is not cancer.

Periductal mastitis

Inflammation with dilation of the subareolar ducts

Mammary duct ectasia

This presents as a perioareolar mass with green-brown nipple discharge

Mammary duct ectasia

Mammary duct ectasia clasically arises in what population?

multiparous postmenopasual women

Cysts that have a blue-dome appearance on gross exam (breast)

Fibrocystic change

How much increased risk for breast carcinoma: apocrine metaplasia

zero increased risk

How much increased risk for breast carcinoma: ductal hyperplasia

2x

How much increased risk for breast carcinoma: sclerosing adenosis

2x

How much increased risk for breast carcinoma: atypical metaplasia

5x

Papillary growth, usually into a large duct (breast)

Intraductal papilloma

This breast pathology classically presents as a bloody nipple discharge in a premenopausal woman

Intraductal papilloma

Histologically what differentiates intraductal papilloma from papillary carcinoma?

Intraductal papilloma contains myoepithelial cells while papillary carcinoma does not

Tumor of fibrous tissue and glands in the breast

Fibroadenoma

Pt presents with a mass in her breast that grows during pregnancy and is painful during menstrual cycle. What is it?

Fibroadenoma

Fibroadenoma-like tumor with overgrowth of fibrous component

Phyllodes tumor

Can phyllodes tumor of breast be malignant?

Yes, but not fibroadenoma

Can fibroadenoma of breast be malignant?

No, but phyllodes tumor can be malignant

Malignant proliferation of cells in ducts with no invasion of the basement membrane

Ductal carcinoma in situ (DCIS)

Ductal carcinoma in situ that extend up the ducts to involve the skin of the nipple

Paget disease of the nipple

What 3 things can show up as calcifications in the breast?

Fat necrosis


Fibrocystic change


Ductal carcinoma in situ

Invasive carcinoma that classically forms duct like structures

Invasive ductal carcinoma

This breast cancer has retraction of the nipple

Invasive ductal carcinoma

What are the 4 subtypes of invasive ductal carcinoma

Tubular carcinoma


Mucinous carcinoma


Medullary carcinoma


Inflammatory carcinoma

This invasive ductal carcinoma has well differentiated tubules that lack myoepithelial cells, what is the prognosis?

Tubular carcinoma, relative good prognosis

This invasive ductal carcinoma has abundant extracellular mucin, what is the prognosis?

Mucinous carcioma, relatively good prognosis

This invasive ductal carcinoma has large, high-grade cells growing in sheets with associated lymphocytes and plasma cells, what is the prognosis?

Medullary carcinoma, relatively good prognosis

This invasive ductal carcinoma has cancer in dermal lymphatics, what is the prognosis?

Inflammatory carcinoma, poor prognosis

Which invasive ductal carcinoma can be mistaken for acute mastitis due to a swollen breast?

Inflammatory carcinoma

Which invasive ductal carcinoma has increased incidence in BRCA1 carriers?

Medullary carcinoma

Malignant proliferation of cells in lobules with no invasion of the basement membrane

Lobular carcinoma in situ (LCIS)

This breast cancer has dyscohesive cells lacking E-cadherin

Lobular carcinoma in situ

Which breast cancer is treated with tamoxifen?

Lobular carcinoma in situ

This invasive carcinoma grows in a single-file pattern, why?

Invasive lobular carcinoma because it lacks E-cadherin

This carcinoma lacks duct formation due to lack of E-cadherinI

Invasive lobular carcinoma

In TNM staging what is the most important prognostic factor?

Metastasis - if there is metastases, very bad

In TNM staging what is the most useful prognostic factor?

Lymph nodes - since most pts do not have metastases this is more useful

What are the three predictive factors in breast cancer and which drugs are they useful for?

Estrogen receptor - responds to tamoxifen


Progesterone receptor - responds to tamoxifen


HER2/neu gene amplification status - responds to trastuzumab

MOA of transtuzumab and what is it used for?

designer antibody directed against HER2 receptor, HER2/neu breast cancer

What is a triple negative breast cancer? what is the significance?

ER, PR, HER2neu negative and it has a poor prognosis

BRCA1 mutation is associated with what cancers?

Medullary breast carcinoma and Serous ovarian carcinoma and Fallopian tube carcinoma

BRCA2 mutation is associated with what cancers?

breast carcinoma in males

What syndrome is associated with male breast cancer?

Klinefelter syndrome

Male breast cancer is most likely which subtype?

invasive ductal carcinoma

Cholecalciferol to 1,25 dihydrocholecalciferol, what organs and what adds 1 and what adds 25 and what order

1st liver adds 25


2nd kidneys add 1

Patient with gynecomastia and small firm testes, abnormally long extremeties. Increased/Decreased: LH,FSH, Testosterone, sperm count

LH,FSH: Increased


Testosterone: Decreased


Sperm count: Decreased


In klinefelter sertoli and leydig cells are destroyed leading to low testosterone. Feedback loop makes LH and FSH increase.

What enzymatic conversion is deficient in biotin deficiency?

Pyruvate to oxaloacetate

Which organ must be tested when pts are using amiodarone?

Thyroid - Serum TSH

Perchlorate and Pertechnate have what MOA and what do they treat?

Block iodide absorption into thyroid by competitive inhibition. Used for hyperthyroidism

MOA of metyrapone test

metyrapone prevents conversion of 11-deoxycortisol to cortisol causing decrease negative feedback loop leading to increase in ACTH in patients with an intact HPA.

What is glucokinase used for?

Insulin receptor in pancreatic beta cells

What GLUT responds most to insulin and in which tissue is it found?

GLUT-4 found in muscles and adipose tissue

Thyroid peroxidase is responsible for what enzymatic reaction?

Oxidation of iodide to iodine


Iodination of thyroglobulin tyrosine


Iodotyrosine coupling of T3 and T4

Which insulins are good for post-prandial? (3)

Lispro


Aspart


Glulisine

Which insulins are good for daily maintenance, once a day? (2)

Glargine


Detemir

Which drug can treat benign prostate hypertrophy as well as male pattern baldness and what is the MOA?

Finasteride - 5a-reductase inhibitor

Neurophysin is an important carrier for what two hormones?

ADH and Oxytocin

Which hypothalamic hormone is inhibited by constant stimulation via dopamine?

Prolactin

MOA of sulfonylureas

Insulin secretagogue

MOA of meglitnides

Insulin secretagogue

Pt is 14 years old and shows no sign of masculinity, he also can't smell. What is the syndrome? what is the defect?

Kallmann syndrome - absence of GnRH secretory neurons

What hormone is the negative feedback for LH? FSH?

LH = testosterone


FSH = Inhibin B

What hormone is the negative feedback for GnRH?

Testosterone

MOA of methimazole

Inhibit thyroid peroxidase centrally does not act peripherally

Name two thionamide drugs and what is a potential complication?

PTU and Methimazole


Agranulocytosis

Which class of heart drugs should not be used with diabetes?

Beta blockers because they mask the adrenergic symptoms of hypoglycemia.

Estrogen does what to thyroid hormone levels and why?

Estrogen increases thyro binding globulin. Which increases the total amount of BOUNDED T4 and T3. This will increase the pool (bound + free) but the free amount will remain the same.

First thing that comes to mind with cryptococcus?

Pigeons

Chagas disease can cause what?

Achalasia

Eosinophils in urine, what is the dx?

acute interstitial nephritis

Neural crest forms what?

PNS

Neural tube forms what?

CNS

Hollow lumen of neural tube forms what?

Ventricles

Low folate levels prior to conception is associated with what?

Neural tube defects

Frog-like appearance of the fetus is associated with?

Anencephaly

Absence of skull and brain is called?

Anencephaly

Maternal polyhydraminos is assocaited with what neural tube defect?

Anencephaly

Failure of posterior vertebral arch to close

Spina bifida

What is spina bifida occulta

Dimple or patch of hair overlying vertebral defect

Congenital stenosis of the channel that drains CSF from the 3rd and 4th ventricles

Cerebral aqueduct stenosis

How does CSF drain starting with lateral ventricle

Lateral


Foramen of Monro


3rd


Cerebral aqueduct


4th


Luschka


subarachnoid space

Congenital failure of the cerebellar vermis to develop

Dandy-Walker Malformation

This presents as a massively dilated 4th ventricle with an absent cerebellum

Dandy-Walker Malformation

Protrusion of meninges is called?

Menigocele

Protrusion of meninges and spinal cord is called?

Meningomyocele

Spina bifida presents with cystic protrusion of the underlying tissue through the vetebral defect, what two cystic protrusions can there be?

Meningocele - protrusion of meninges


Meningomyelocye - protrusion of meningees and spinal cord

Congenital extension of cerebellar tonsils through foramen magnum

Arnold-Chiari Malformation

Hydrocephalus can occur with what two neural tube defects?

Cerebral aqueduct stenosis


Arnold-Chiari Malformation

Cystic degeneration of the spinal cord

Syringomyelia

Syringomyelia may arise with what malformation?

Arnold-Chiari malformation

This presents as loss of pain and temperature sensation in the upper extremities

Syringomyelia

Syringomyelia is due to involvement of what portion of the spinal cord?

Anterior white commisure

Syringomyelia may lead to what syndrome if the lateral horn of the hypothalamospinal tract is involved?

Horner


ptosis


miosis


anhidrosis

Damage to the anterior motor horn due to poliovirus infection

Poliomyelitis

Inherited degeneration of anterior motor horn

Werdnig-Hoffman disease

Werdnig-Hoffman disease has what inheritance pattern?

Autosomal recessive

Presents as floppy baby; death occurs within a few years after birth. Congenital disease.

Werdnig-Hoffman Disease

Lowor motor neurons is controlled by what part of the spinal cord?

Anterior motor horn

Upper motor neurons is controlled by what part of the spinal cord?

Lateral corticospinal tract

Spastic paralysis with hyperreflexia, increased muscle tone, and positive Babinski sign is LMN or UMN disorder?

UMN

Flaccid paralysis with muscle atrophy, fasiculations, weakness and decreased muscle tone, and negative babinski sign is LMN or UMN disorder?

LMN

What distinguished ALS from syringomyelia?

ALS does not have sensory impairment

Familial ALS is associated with a mutation in what?

Zinc-Copper superoxide dismutase

Degenerative disorder of cerebellum and spinal cord tracts

Friederich Ataxia

Inheritance pattern of Friederich Ataxia

Autosomal Recessive

Unstable trinucleotide repeate (GAA) in frataxin gene describes what disorder?

Friedereich ataxia

Which spinal cord lesion is associated with hypertrophic cardiomyopathy?

Friederich ataxia

Meningitis is inflammation of what?

Leptomeninges

Leptomeninges is composed of what?

Pia and arachnoid, NOT DURA

3 most common organism to cause meningitis in neonates

Group B Strept


E. Coli


Listeria Monocytogenes

most common organisms to cause meningitis in children and teenagers

N Meningitidis

most common organisms to cause meningitis in adults and elderly

Strep pneumo

most common organism to cause meningitis in nonvaccinated infants

H flu

Most common organism to cause meningitis in immunocompromised?

Fungi

Most common viral cause of meningitis?

Coxsackievirus

Where are lumbar punctures done? why?

L4-L5 , spinal cord ends at L2 but the subarchnoid space and cauda equina ends at S2

With a lumber puncture what are you piercing and where do you stop at?

Skin, ligament, epidural space, dura, arachnoid, DO NOT PIERCE PIA

LP with neutrophils with low glucose suggest what cause of meningitis?

Bacterial

LP with lymphocytes with normal CSF glucose suggest what cause of meningitis?

Viral

LP with lymphocytes with low CSF glucose suggests what cause of meningitis?

Fungal

Complications with meningitis is usually seen with what type of meningitis?

Bacterial

In moderate global cerebral ischemia what are the highly vulnerable regions?

Pyramidal neurons of cerebral cortex - layers 3,5,6 (laminar necrosis)


Pyramidal neurons of hippocampus


Purkinje layer of cerebellum

Ischemia of pyramidal neurons of cerebral cortex damages what layers and leads to what type of necrosis?

Layers 3,5,6, laminar necrosis

This type of stroke is due to rupture of an athersclerotic plaque

Thrombotic stroke

Thrombotic strokes usually have what kind of infarct?

Pale infarct

This type of stroke is due to thromboemboli

Emoblic stroke

Embolic stroke usually have what kind of infarct?

Hemorrhagic Infarct

This stroke occurs secondarily to hyaline arteriosclerosis, a complication of HTN.

Lacunar stroke

Which vessels are most commonly involved in lacunar strokes?

lenticulostriae vessels

Ischemic strokes result in what kind of necrosis?

Liquefactive

Stages of Ischemic stroke , 1 day, 1 week, 1 month

Red neurons | Neutro then Mircoglial | Granulation Tissue | Cyst (gliosis)

What is the most common site for intracerebral hemorrhage?

Basal ganglia

Charcot-Bouchard microaneurysms are associated with what?

Intracerebral hemorrhage

Pt presents with worst headache of my life, what is the dx?

Subarachnoid hemorrhage

Bleeding on the bottom of the brain, what is the dx?

Subarachnoid hemorrhage

Lumbar puncture shows xanthochromia (yellow hue to bilirubin breakdown) what is the dx?

Subarachnoid hemorrhage

Most common cause of a subarachnoid hemmorrhage?

Berry aneurysm

What is the most frequent location of berry aneurysm?

anterior circle of willis (branch points of anterior communicating artery)

What are berry aneurysms associated with?

Marfan and ADPKD

Why is it easy for berry aneurysms to rupture?

they lack a media layer

Collection of blood between the dura and the skull

Epidural Hematoma

Epidural hematoma are usually due to what?

Fracture of temporal bone with rupture of middle meningeal artery

This head trauma has lucid intervals that may precede neurologic signs "talk and die"

Epidural hematoma

Collection of blood underneath the dura; blood covers the surface of the brain.

Subdural hematoma

Subdural hematomas are usually due to what?

tearing of bridging vein between dura and arachnoid

Epidural and subdural hematoma has what lethal complication?

Herniation

Displacement of brain tissue due to mass effect or increased intracranial pressure

Herniation

Name the herniation and the complication that arises from it: displacement of cerebellar tonsils into the foramen magnum

Tonsillar herniation: compression of brain stem leads to cardiopulmonary arrest

Name the herniation and the complication that arises from it: Displacement of cingulate gyrus under the falx cerebri

Subfalcine herniation: compression of anterior cerebral artery leads to infarction

Name the herniation and the complication that arises from it: displacement of temporal lobe uncus under the tentorium cerebelli

Uncal Herniation


Compression of cranial nerve III (oculomotor), eye moves down and out and a dilate pupil


Compression of posterior cerebral artery leads to infarction of occipital lobe (contralateral homonymous hemianopsia)


Rupture of the paramedian artery leads to Duret (brainstem) hemorrhage


Deficiency of arylsulfatase leads to what disorder?

Metachromatic leukodystrophy

Metachromatic leukodystrophy has a deficiency in what? and what does this lead to?

Arylsulfatase - myelin cannot be degraded; accumulates in lysosomes

Deficiency of galactocerbroside B-galactosidase leads to what disorder?

Krabbe disease - a form of leukodystrophy

Krabbe disease has a deficiency in what? and what does this lead to?

galactocerbroside B-galactosidase - galactocerebroside accumulates in macrophages

Impaired addition of coenzyme A to long-chain fatty acids leads to what disorder?

Adrenoleukodystrophy

Adrenoleukodystrophy has what deficiency? what does this lead to?

Impaired addition of coenzyme A to long-chain fatty acids - fatty acids accumulate damaging adrenal gland and white matter

Autoimmune destruction of CNS myelin and oligodendrocytes

Multiple Sclerosis

Multiple Sclerosis is associated with which HLA?

HLA-DR2

What disease is more commonly seen in regions away from the equator?

Multiple sclerosis

What is internuclear ophthalmoplegia and what is it associated it with?

Multiple sclerosis - when you try to move the eyes to one side only one eye moves because of a defect in the medial longitudinal fasciculus

LP of multiple sclerosis would show what?

Oligoclonal IgG bands

Oligoclonal IgG bands are found in what CNS disorder?

Multiple sclerosis

How do you slow the progression of multiple sclerosis?

Interferon beta

Progressive, debilitating encephalitis leading to death

Subacute sclerosing panencephalitits

What is subacute sclerosing panencephatlitis due to?

persistent infection of the brain by measles virus

Persistent infection of the brain by measles virus leads to what?

Subacute sclerosing panencephalitis

JC virus infection of oligodendrocytes leads to what?

Progressive multifocal leukoencephalopathy

Progressive multifocal leukoencephalopathy is due to what?

JC virus infection of oligodendrocytes

Focal demyelination of pons

Central Pontine Myelinolysis

Rapid IV correction of hyponatremia can cause what?

Central pontine myelinolysis

What CNS disorder presents with acute paralysis giving a "locked in" syndrome?

Central Pontine Myelinolysis

Degeneration of brainstem and basal ganglia leads to what kind of disorders?

Movement disorders

E4 apolipoprotein E associated with increased or decreased risk of alzheimer

Increased - because more ABeta-protein is being made

E2 apolipoprotein E associated with increased or decreased risk of alzheimer?

Decreased

Familial cases of early alzheimers associated with what?

Presenelin 1

Why is there early onset alzheimers in down syndrome patietns?

Amyloid precurrsor protein (APP) is on chromosome 21. Increased APP can increase amount converted into Abeta amyloid which will cause down syndrome.

Hydrocephalus ex vacuo - what is it and what is it associated with?

Dilated ventricles due to atrophy of brain, associated with alzheimer

AB amyloid depositing around blood vessels in the brain: what is it called and what is the consequence?

Cerebral amyloid angiopathy - increased likelihood of rupture into brain.

What are neurofibrillary tangles composed of? what is it associated with?

Composed of hyperphosphorylated Tau, associated with Alzheimer Disease

What type of protein is Tau?

Microtubule associated protein - hyperphosphoyrlated Tau assocaited with Alzheimer

Multifocal infarction and injury of the brain due to HTN, atherosclerosis, or vasculitis

Vascular dementia

Degenerative disease of FRONTAL and TEMPORAL cortex; spares parietal occipital lobes

Pick Disease

Pick Disease primarily affects which portion of the brain?

Frontal and Temporal, spares parietal and occiptal lobes

Round aggregates of tau protein is associated with what brain disease?

Pick disease


Alzheimer has neurofibrilary tangles assocaited with tau

Parkinson disease has a degenerative loss of what type of neurons where?

Dopaminergic neurons - substantia nigra pars compacta of the basal ganglia

Contamination of illicit drugs with MPTP is associated with what disorder?

Parkinson Disease

What are Lewy bodies associated with and what are they?

Parkinson's disease - round eosinophilic inclusions of alpha-synuclein

Parkinson with early dementia is called what? how do you distinguish them?

Lewy body dementia - the lewy bodies are found in the cortex instead

Degeneration of GABAergic neurons in the caudate nucleus of the basal ganglia.

Huntington Disease

Inheritance pattern for Huntington

Autosomal disorder

Pathophysiology of chorea in Huntington disease

Degeneration of GABAergic neurons leads to loss of inhibition from the caudate nucleus of the basal ganglia to the cortex. This results in random movement.

What is the expanded trinucleotide repeat in the huntingtin gene (what is the actual code)?

CAG

Further expansion of the trinucleotide repeat in huntington disaese happens where/when?

Spermatogenesis

Increase CSF resulting in dilated vdentricles

Normal Pressure Hydrocephalus

What is the triad for normal pressure hydrocephalus?

Wet, Wacky, Wobbly


Urinary incontinence


Dementia


Gait instability


What are the two treatments for normal pressure hydrocephalus

Lumbar puncture, ventriculuperitoneal shunting.

Degenerative disease due to prion protein

Spongiform Encephalopathy

Pathophysiology of Spongiform encephalopathy

Prion proteins (PrPc) in the alpha helical form being converted to Prion proteins in the Beta pleated (PrPsc).

Creutzfeldt-Jakob disease presents with what kind of dementia?

Rapidly progressive dementia

Familial fatal insomnia is what type of CNS disease?

Spongiform encephalopathy

Metastatic brain cancer usually come from where?

Lung, Breast, Kidney

Brain tumors in adults usually arise where relative to tentorium?

Supratentorial - adults


Infratentorial - kids

Malignant, high-grade tumor of astrocytes

Gliobastoma Multiforme - Normally found in adults, most common malignant CNS tumor in adults

What is the most common malignant CNS tumor in adults

Glioblastoma Multiforme

Which CNS tumor crosses the corpus callosum?

Glioblastoma Multiforme

Which CNS tumor is GFAP positive? (2)

Glioblastoma Multiforme and Pilocytic Astrocytoma

Benign tumor of arachnoid cells

Meningioma - most common benign CNS tumor in adults

Meningioma is most common in what poppulation?

Adult Females

Which CNS tumor has a whorled pattern on histology

Meningioma

Which CNS tumor has psammoma bodies

Meningioma

Benign tumor of Schwann cells

Schwannoma

Bilateral schwannomas of cranial nerve VIII is seen in what disorder?

Neurofibromatosis 2

Which CNS tumor is S-100 positive?

Schwannoma

Malignant tumor of oligodendrocytes

Oligodendroglioma

Calcified tumor in white matter of the CNS

Oligodendroglioma

Which CNS tumor has a fried egg appearance on biopsy?

Oligodendroglioma

Which CNS tumor involves the frontal lobe that may present with seizures?

Oligodendroglioma

Benign tumor of astrocytes

Pilocytic astrocytoma

Most common benign brain tumor in children

Pilocystic astrocytoma

This CNS tumor reveals a cystic lesion with a mural nodule

Pilocystic astrocytoma

Biopsy of this tumor shows Rosenthal fibers, what is the tumor and what are rosenthal fibers?

Pilocystic astrocytoma - rosenthal fibers are thick eosinophilic granular bodies

Malignant tumor derived from granular cells of cerebellum

Medulloblastoma

What are drop metastases and what is it associated with? (CNS)

Metastasis to cauda equina - Medulloblastoma

Malignant tumor of ependymal cells

Ependymoma

Which CNS tumor most commonly arises in 4th ventricle, may present with hydrocephalus.

Ependymoma

Which CNS tumor can present with bitemporal hemianopsia?

Craniopharyngioma

What is a Brenner tumor?

Surface epithelial tumor of the ovary that contains urothelium

What prostaglandin analogue treats glaucoma?

Latanaprost

MOA of Latanaprost and what does it do?

Prostaglandin analogue that treats glaucoma

What are streak ovaries associated with?

Turners syndrome

Marcophages express this surface receptor

CD 14

CNS tumors that stain with synaptophysin generally originate from what cell?

Neurons

What amino acids must be restricted in Maple Syrup Urine Disease?

leucine, isoleucine, valine

Name this immature defense: Tantrums

Acting out - expressing unacceptable feelings and though through actions

Name this immature defense: Extreme forms can result in dissociative identity disorders

Dissociation - Temporary, drastic change in personality, memory, consciousness, or motor behavior to avoid emotioanl stress

Name this immature defense: a common reaction to newly diagnosed AIDS and cancer patients

Denial - Avoiding the awareness of some painful reality

Name this immature defense: mother yells at her child because her husband is yelling at her

Displacement - transferring avoided ideas and feelings to some neutral person or object

Name this immature defense: Men fixating on sports games

Fixation - partially remaining at a more childish level of development

Name this immature defense: Abused child identifies with an abuser

Identification - Modeling behavior after another person who is more powerful

Name this immature defense: Describing murder in graphic details with no emotional response

Isolation (of affect) - separating feeling from ideas and events

Name this immature defense: A man who wants another woman thinks his wife is cheating on him

Projection - attributing an unacceptable internal impulse to an external source

Name this immature defense: After getting fired, claiming that the job was not important anyway.

Rationalization - proclaiming logical reasons for actions actually performed for other reasons, usually to avoid self-blame

Name this immature defense: A patient with lustful thoughts enters a monastery.

Reaction formation - replacing a warded-off idea or feeling by an emphasis on its opposite

Name this immature defense: Seen in children under stress such as illness, punishment, or birth of a new sibling (bedwetting in a previously toilet-trained child when hospitilized)

Regression - turning back the maturational clock

Name this immature defense: Not remembering a conflictual or traumatic experience; pressing bad thoughts into the unconscious

Repression - involuntary withholding an idea or feeling from conscious awareness

Name this immature defense: A patient says that all nurses are cold and insensitive but that the doctors are warm and friendly.

Splitting - believing that people are either all good or all bad at different times due to intolerance of ambiguity.

Name this mature defense: Mafia boss makes large donation to charity

Altruism

Name this mature defense: Nervous medical student jokes about the boards

Humor

Name this mature defense: Teenager's aggression toward his father is redirected to perform well in sports

Sublimation

Name this mature defense: Choosing to not worry about the big game until it is time to play

Suppression

Achondroplasia is due to what kind of mutation and where? What does this mutation cause?

Activating mutation in FGFR3 - over expression of FGFR3 inhibits growth.

Inheritance pattern for achondroplasia

Autosomal dominant

Autosomal dominant defect in collagen type 1 syntehsis

Osteogeneis Imperfecta

Inherited defect of bone resorption resulting in abnormally thick, heavy bone that fracture easily

Osteopetrosis

Carbonic anhydrase II mutation can lead to what disorder?

Osteopetrosis

Defective mineralization of osteoid due to low levels of vitamin D

Rickets/Osteomalacia

Rachitic rosary - deposoition of osteoid at the constochondral junction is associated with what disease?

Rickets

How do you distinguish osteomalacia from osteoporosis?

Lab values


Osteomalacia has low calcium, phosphate, increased PTH and alkaline phosphatase. In Osteoporosis everything is normal

Most feared complication of Paget Disease of Bone

Osteosarcoma

Which bone disease can cause high-output cardiac failure

paget disease - creation of AV shunts in bone

Most common location for osteomyelitis in the bones for children? adults?

children - metaphysis


adults - epiphysis

Most common bacteria to cause osteomyelitis?

S. aureus

This bone tumor pain resolves with aspirin

Osteoid Osteoma

Benign tumor of osteoblasts surrounded by rim of reactive bone

Osteoid osteoma

What differentiates osteoblastoma from osteoid osteoma?

Greater than 2cm and does not respond to aspirin

Most common benign tumor of bone

Osteochhondroma

Malignant proliferation of osteoblasts

Osteosarcoma

Risk factors for osteosarcoma (3)

Familial retinoblastoma


Paget disease


Radiation exposure

Which portion of bone do osteosarcomas normally arise?

Metaphysis of long bones

Which bone tumor arises in the epiphysis?

Giant Cell Tumor

Which bone tumor is comprised of multnucleated giant cells and stromal cells

Giant Cell tumor

Soap bubble appearance on X-ray suggests what bone tumor?

Giant Cell Tumor

Malignant proliferation of poorly-differentiated cell derived from neuroectoderm

Ewing Sarcoma

This bone tumor usually arises in the medulla of small bones of the hands and feet

Chondroma

This bone tumor normally arises in medulla of the pelvis or central skeleton

Chondrosarcoma

Ewing sarcoma is derived from what germ cell?

Neuroectoderm

What is the translocation for ewing sarcoma?

11;22

Which bone tumor has onion-skinning on x-ray?

Ewing sarcoma

Joint surfaces are lined by hyalin cartilage, what type of collagen is this?

Type II

This arthritis presents with stiffness in the morning that gets worst during the day

Osteoarthritis

This arthritis affects DIP and PIP (distal and proximal interphalagnes)

Osteoarthritis

What are Herbeden and Bouchard nodes?

Herberden nodes = osteophytes on DIP


Bouchard nodes = osteophytes on PIP


Found in osteoarthritis

Rheumatoid arthritis is associated with which HLA?

HLA-DR4

What is the hallmark of rheumatoid arhtritis?

Synovitis leading to formation of pannus (inflamed granulation tissue)

This arthritis presents with morning stiffness that improves with activity

Rheumatoid arthritis

This arthritis only involves PIP leading to swan-neck deformity

Rheumatoid arthritis

What is swelling of bursa behind the knee associated with?

Rheumatoid arthritis - this is called a baker cyst

This pathology presents with IgM autoantibody against Fc portion of IgG

Rheumatoid Arthritis - Rheumatoid Factor

Rheumatoid arthritis presents with this immulogical finding

Rheumatoid factor - IgM autoantibody against Fc portion of IgG

What are the two complications that can arise from Rheumatoid arthritis?

anemia of chronic disease - chronic inflammation increases hepcidin


secondary amyloidosis - SAA gets made and converted to AA leading to amyloidosis

Seronegative spondyloarthritis is characterized by what 3 things?

Lack of rheumatoid factor


Axial involvement - spondylo


HLA-B27 assocaition

What is the triad for Reiter syndrome

Can't see, can't pee, can't climb a tree


-arthritis


-urethritis


-conjunctivits

Involves axial and peripheral joints; DIP joints of hands and feet are most commonly affected, leading to sausage finger or toes

Psoriatic arthritis

Arthritis due to an infectious agent, usually bacterial

Infectious arthritis

Most common cause for infectious arthritis (organism)

N gonorrhoeae

Deposition of monosodium urate crystals in tissues, especially joints

Gout

3 causes for secondary gout

Leukemia and myeloproliferative disorders


Lesch-Nyhan syndrome


Renal insufficiency

How does leukemia and myeloproliferative disroders cause gout?

Increased cell turnover leads to hyperuricemia

How does Lesch-Nyhan syndrome cause gout?

Deficiency in hypoxanthine-guanine phosphoribosyltransferase (HGPRT) leads to increased xanthine and uric acid since hypoxanthine and guanine can no longer be salvaged.

3 parts of Lesch-Nyhan syndrome

Self-mutilation


Gout


Mental retardation

How does renal insufficiency cause gout?

Kidneys can no longer excrete uric acid

What is podagra?

Painful arthritis of the great toe caused by acute gout

What are the two things that can cause acute gout?

Alcohol or consumption of meat

What are tophi? and what are they assocaited with?

Uric acid crystals in soft tissue or joints - Chronic Gout

Chronic gout can lead to what two things?

Tophi - uric acid crystals in soft tissue or joints


Renal failure - urate cyrstals deposit in kidney tubules

This pathology presents with synovial fluid showing needle shaped crystals with negative birefringence under polarized light

Gout

This pathology presents with synovial fluid showing rhomboid-shaped crystals with weakly positive biirefrigence under polarized light

Pseudogout

Pseudogout is due to deposition of what?

Calcium pyrophosphate

Inflammatory disorder of the skin and skeletal muscle

Dermatomyositis

It a pt presents with dermatomyositis what should you be worried about?

Underlying carcinoma especially gastric carcinoma

What is the hallmark of dermatomyositis

Bilateral proximal muscle weakness

What two disease can have malar rash?

SLE and Dermatomyositis

When muscle is damaged what is elevated in blood?

Creatine kinase

What diseases have a positive ANA? (2)

Lupus and Dermatomyositis

Anti-Jo-1 antibody is indicative of what disease?

Dermatomyositis

Perymysial inflammation is found in what?

Dermatomyositis - perymysial (close to the skin)


Polymyositis = endomysial

Endomysial inflammation is found in what?

Polymyositis = endomysial


Dermatomyositis = perimysial

Inflammatory disorder of skeletal muscle without skin involement

Polymyositis

Degenerative disorder characterized by muscle wasting and replacement of skeletal muscle with adipose tissue

X-Linked Muscular dystrophy

In X-linked muscular dystrophy what is the skeletal muscle replaced with?

Adipose tissue

Deletions in what gene is responsible for Duchenne muscular dystrophy

Dystrophin

What is the difference between x-linked muscular dystrophy and Duchenne?

X-linked = mutated dystrophin


Duchenne = deletion of dystrophin

What causes becker muscular dystrophy?

mutation of dystrophin

Calf pseudohypertrophy is characteristic finding of what disease?

Muscular dystrophy

Autoantibodies against the postsynaptic acetylcholine receptor of NMJ

Myasthenia gravis

What is the pathophysiology of myasthenia gravis

Autoantibodies against the postsynaptic acetylcholine receptor of NMJ

What type of drugs are used for myastehnia gravis?

anticholinesterase agents

This is associated with thymic hyperplasia or thymoma; thymectomy improves symptoms

Myasthenia Gravis

Antibodies against presynaptic calcium channels of the NMJ

Lambert-Eaton syndrome

Myasthenia gravis classically involves what part of the body?

Eyes

Myasthenia gravis clasically involves the eyes, what syndrome spares the eye?

Lambert-Eaton

Lambert-Eaton vs Myasthenia Gravis = which improves with use, which does not?

Myasthenia Gravis = does not


Lambert-Eaton = improves

Most common benign soft tissue tumor in adults

Lipoma

What is the characteristic cell of liposarcoma?

Lipoblast

Most common malignant soft tissue tumor in adults

Liposarcoma

Cardiac rhabdomyoma is associated with what?

Tuberous sclerosis

Most common malignant soft tissue tummor in children?

Rhabdomyosarcoma

What is the classic site for rhabdomyosarcoma in young girls?

Vagina

Atopic dermatitis (Eczema) is what type of hypersensitivity?

Type 1 - associated with asthma and allergic rhinitis

Contact dermatitis is what type of hypersensitivity?

Type 4

What bacteria is responsible for acne vulgaris?

Propinibacterium acnes

What is psoriasis due to?

excessive keratinocyte proliferation

What is acanthosis?

Epidermal hyperplasia

What is auspitz sign and what is it associated with?

Thinning of epidermis above elongated dermal papillae; resulting in bleeding when scale is picked off. Psoriasis

What is lichen planus associated with?

hepatitis C virus infection

Autoimmune destruction of desmosomes between keratinocytes

Pemphigus vulgaris

What is pemphigus vulgaris due to?

IgG antibody against desmoglein

What type of hypersensitivity is pemphigus vulgaris?

Type II

Autoimmune destruction of hemidesmosomes between basal cells and the underlying basement membranes

Bullous pemphigoid

What is bullus pemphigoid due to?

IgG antibody against hemidesmosome components of the basement membrane

Autoimmune deposition of IgA at tips of dermal papillae

Dermatitis Herptiformis

Hypersensitivity reaction characterized by targetoid rash and bullae.

Erythema Multiforme

What is erythema multiforme most commonly associated with?

HSV infection

What is Steven Johnson Syndrome?

Erythema multiforme with oral mucosa/lip involvement and fever

What is the severe form of steven johnson syndrome?

Toxic Epidermal Necrolysis

Most common cause for toxic epidermal necrolysis?

adverse drug reaction

Benign squamous proliferation common tumor in elderly

Seborrheic keratosis

Epidermal hyperplasia with darkening of the skin

Acanthosis Nigricans

What is acanthosis nigricans associated with? (2)

insulin resistance and malignancy

What is the most common cutaneous malignancy?

Basal cell carcinoma

Xeroderma pigmentosum what is the defect?

can not repair DNA damage so pyrimidine dimers persist

What is the most common cancer to arise from immunosuppresive therapy?

Squamous cell carcinoma

What malignancy is associated with arsenic poisoning?

Squamous cell carcinoma

well differentiatned squamous cell carcinoma that develops and rapidly regresses

Keratoacanthoma

Where are melanocytes derived from?

Neural crest

Localized loss of skin pigmentation due to autoimmune destruction of melanocytes

Vitiligo

Albinism is usually due to what?

Enzyme defect (usually tyrosinase) that impairs melanin production

Freckle is due to increased of what?

Increased in melanosomes not melanocytes

Benign neoplasm of melanocytes

Nevus (mole)

Most common mole in children

Junctional nevus

Junctional nevus that grows into the dermis is called?

compound nevus

Most common mole in adults

Intradermal nevus

What is the progression for moles?

Junctional nevus becomes compound becomes intradermal

What are the ABCD of Melanoma?

Assymetry


Border are irregular


Color is not uniform


Diameter > 6mm

What is the most important prognostic factor in melanoma?

Depth of extension (Breslow thickness)

Name the melanoma: radial growth, lentiginous proliferation

Lentigo maligna melanoma

Name the melanoma: early radial growth

Superficial spreading

Name the melanoma: early vertical growth

nodular

Most common type of melanoma

superficial spreading

What is impetigo most often due to? (2 organisms)

S aureus or S pyogenes

What is cellulitis most often due to? (2 organisms)

S aureus or S pyogenes

What is crepitus and what is it associated with?

When you can hear crackling of air underneath the skin, associated with necrotizing fascitis

Sloughing of skin with erythematous rash and fever

Staphylococcal Scalded Skin Syndrome

What bacteria causes Scalded Skin Syndrome? what is the pathophysiology?

Staphylococcal Aureus release exfoliative A and B toxin resulting in epidoermolysis of the stratum granulosum

Difference between toxic epidermal necrolysis and scalded skin syndrome in regard to layers

TEN = dermal-epidermal junction


Scalded Skin Syndrome = Stratum granulosum

Firm, pink, umbilicated papules due to poxvirus

Molluscum Contagiosum

Name the melanoma: arises on palm and or soles, often in dark-skinned individuals

Acral Lentigerous

Exposure to sunlight allows conversion of what to what in Vitamin D metabolism?

7-dehydrocholesterol to cholecalciferol

2 years of mild depression is called what?

Dysthymic disorder

Ehlers-Danlos syndrome is caused by a defect in what?

Collagen formation

Marfan syndrome is caused by a defect in what?

Fibrillin 1

Congenital hypothyroidism presents with what 4 features?

Umbilical hernia


Macroglossia


Hypotonia


Jaundice


What is caudal regression syndrome associated with? Poorly developed lumbar spine and sacrum at birth.

Maternal diabetes

Young football player suddenly dies from splenic rupture what is the dx?

Infectious mononucleosis caused by Epstein Barr Virus

Bamboo spine associated with what?

Ankylosing spondylitis

CD4+ vs CD8+ for Polymyositis and dermatomyositis

Dermatomyositis = CD4+


Polymyositis = CD 8+

Crest is associated with what antibody?

Anti-centromere

What is CREST?

Calcinosis


Raynaud Phenomenon


Esophagueal dysmotility


Sclerodactyly


Teangiectasia

What does leukotriene B4 do?

Neutrophil chemotactic

What does PGI2 do? (2)

inhibits platelet aggregation and promotes vasodilation

MOA of celecoxib

Cox-2 ihibitor

MOA of kettorolac

NSAID

MOA of Diclofenac

NSAID

MOA of allopurinol and what is it used for?

Inhibits xanthine oxidase - Gout

MOA of febuxostat and what is it used for?

Inhibits xanthine oxidase - gout

MOA of colchicine and what is it used for?

Stabilizes tubulin which inhibits microtubule polymeration impairing leukocyte chemotaxis and degranulation - gout

MOA of etanercept and what is it used for?

Etanercept is a TNF decoy RECEPTOR - used for rheumatoid arthritis, psoriasis

MOA of adalimunmab

Anti-TNFa antibody - IBD, rheumatoid, ankylosing spondylitits, psoriasis

MOA of Probenecid and what is it used for?

Inhibits reabsorption of uric acid in PCT

Pts with retinoblastoma have increased risk of what other kind of cancer?

Osteosarcoma

Influenza vaccine works how?

Antibodies against hemagluttinin

Which vein is used in CABG?

Saphenous vein

Progressive onset of CHF over many years due to chronic ischemic myocardial damage

Chronic Ischemic Heart Disease

Death from cardiac causes within 1 hr usually due to lethal arryhtmia

Sudden Cardiac Death

How does sudden cardiac death cause death?

Lethal arrhthmia

Which part of the HPA do long term corticosteroids suppress?

The whole thing. ACTH, CRH, Cortisol are all decreased after long term use.

In Nieman-Pick disease what enzyme is deficient?

Sphingomyelinase

How do you tell the difference between Vit B12 and Folate deficiency?

Deficiency Vit B12 causes neurologic problems.

Aldose reductase turns glucose into sorbitol, what is the next enzyme and the next sugar?

Sorbitol dehydrogenase into Fructose

Low alpha-fetoprotein in utero is associated with what?

Trisomy 21

Increased alpha-fetoprotein in utero is associated with what?

multiple gestation


neural tube defects


abdominal wall defects

What is cyanocobalamin?

Vit B12

Thrombophlebitis at various sites may indicate what?

Visceral malignancy

Anorexia has what effect on hormones?

Loss of pulsatile release of GnRH

Where are serotenergic neurons found in the brain?

Raphe nuclei

What is used as a chemoprophylaxis for N. meningitidis infections?

Rifampin

Overuse of vitamin A can cause what?

Hepatosplenamegaly


Intracranial HTN


Skin changes

What is the antibiotic of choice for rickettsial diseases?

Doxycycline

What does PT stand for and what pathway is it?

Protrombin Time - Extrinsic

What does aPTT stand for and which pathway is it?

activated partial thromboplastin time - Intrinsic

Eukaryotes and prokaryotes have similar replication times but eukaryotic DNA is much larger. How do eukaryotes speed up DNA synthesis?

Multiple origins of replication

What is the most common cause for E. Coli bactermeia?

UTI

What is the DOC for absence seizures and associated tonic-clonic seizures?

Valproate

What can treat absence seizures but not tonic-clonic seizures?

Ethosuximide

Isoniazid has what toxicity?

Hepatic toxicity

Pt presents with jugular vein distension, pulsus paradoxus, and tachycardia. What's the dx?

Cardiac tamponade

Supine dyspnea that is relieved by sitting up is specific for what pathology?

Left sided heart failure -