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1023 Cards in this Set
- Front
- Back
Breast enlargement during pregnancy is an example of
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Hyperplasia
|
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What about skeletal muscle enlargement from weight lifting
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Hypertrophy
|
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Atrophy is often marked by the presence of
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Autophagic granules
|
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Metaplasia (eg squamous metaplasia of the bronchi from long-term smoking) is often associated with
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Chronic irritation
|
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Metaplasia can also be associated with
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Vitamin A deficiency
|
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Myeloid metaplasia is aka
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Extramedullary hematopoiesis
|
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____ is the most common cause of hypoxic cell injury
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Ischemia (obstruction of arterial blood flow)
|
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Describe the sequence of early stage hypoxic cell injury
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Mitochondria effected, decreased ATP production, failure of Na-K-ATPase pump, retained Na+ causes swelling of ER, diasaggregation of ribosomes and ↓protein synthesis, ↑glycolysis and ↑lactate
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Name two markers of late-stage cell hypoxia
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Myelin figures and cell blebs
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What signifies the point of no return in cell damage
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Calcium influx, calcification of the mitochondria
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How long can neurons, muscle cells, cardiac cells and hepatocytes last without oxygen
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N 3-5 minutes (Purkinjes in cerebellum and neurons of the hippocampus most sensitive)
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Name two common conditions where oxygen toxicity is due to free radical damage
|
Retrolental fibroplasia – retinopathy of preemies
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Name the classic ultrastructure markers of barbiturate poisoning
|
Proliferation and hypertrophy of the SER of the hepatocyte
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Reperfusion following _____ injury is also a major cause of free radical injury
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Ischemic
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Name the intracellular enzymes involved in free radical degradation
|
Glutathione peroxidase, catalase, and superoxide dismutase
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Which vitamins are significant anti-oxidants
|
Vit A, C and E
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Chemical cellular injury is modeled by _____
|
Hepatocyte injury due to CCl4
|
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Briefly discuss this model:
|
CCl4 is processed by P-450 enzymes in the SER, this produc s CCL3-, which initiates lipid peroxidation of intracellular membranes, dissagre-gation of ribosomes, ↓protein synthesis including lipoproteins, thus fatty change occurs
|
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Ultimately, the _____ membrane is damaged due to the lipid perox. of SER, this results in
|
Massive calcium influx with resultant mitochondria damage and cell death
|
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Necrosis only occurs within
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Living organisms
|
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______ necrosis most often occurs from a sudden cutoff of blood supply to an organ, particularly end-organs like the heart and kidney
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Coagulative
|
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Pathological changes involved?
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General architecture well preserved, except for nuclear changes
|
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_____ is chromatin clumping w/↑basophilia
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Pyknosis
|
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_____ is fragmentation of DNA
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Karyorrhexis
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_____ is the fading of chromatin material
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Karyolysis
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Enzymatic liquefaction of necrotic tissue, most often in the ____ is aka liquefactice necrosis
|
|
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Where else does liquefactive necrosis occur
|
In bacterial infections
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_____ necrosis shares features of both coag. And liquefactive necrosis. Most commonly seen in TB granulomas
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Caseous
|
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Which necrosis results from interruption of blood supply to a lower extremity or bowel
|
Gangrenous
|
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When is it called WET gangrene
|
When it is complicated by infective heterolysis and consequent liquefactive necrosis
|
|
Fibrinoid necrosis is marked by
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Deposition of fibrin-like proteinaceous material in arterial walls
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It is often associated with
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Immune mediated vascular damage (vasculitis)
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Fat necrosis occurs in what two forms
|
Traumatic fat necrosis
|
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Traumatic follows severe injury to tissue with high fat content, whereas enzymatic is a complication of
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Acute hemmorrhagic pancreatitis, where proteolytic and lipolytic enzymes seep into pancreatic parenchyma and digest it, which liberates fatty acids that are then saponified
|
|
Which has an inflammatory rxn?
|
|
|
Fatty change (aka _____) is characterized by
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Steatosis, accumulation of intracellular parenchymal TGs.
|
|
Most frequently observed in which organs?
|
Liver, heart and kidney
|
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_____ change describes a homogenous, glassy eosinophilic appearance
|
Hyaline
|
|
Argyria is aka _____
|
Silver poisoning, may cause a permanent gray discoloration of the skin and conjunctivae
|
|
Decreased melanin pigmentation is seen in what two conditions
|
Albinism and vitiligo
|
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Bilirubin is a catabolic product of both _____ and _____
|
Hemoglobin and methemoglobin
|
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What is hemosiderin?
|
Iron containing pigment that consists of aggregates of ferritin
|
|
Stains best with _____
|
Prussian blue
|
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Systemic hemosiderosis is hemosiderin deposition without without _____
|
Tissue or organ damage
|
|
May result from?
|
Hemorrhage, blood transfusions, hemolysis, excessive dietary iron
|
|
How is hemoCHROMATOSIS different
|
Damage to many tissues and organs, scarring and organ dysfunction
|
|
Manifests itself as?
|
Hepatic cirrhosis and pancreatic fibrosis, leading to diabetes mellitus
|
|
Most often caused by
|
Hereditary disorder characterized by ↑iron absorption
|
|
Caused by a mutation in which gene?
|
Hfe gene on chromosome 6
|
|
Organs most affected?
|
Liver pancreas and myocardium
|
|
Triad of findings?
|
Micronodular cirrhosis, diabetes mellitus and skin pigmentation (bronze diabetes)
|
|
Which pts are at greatest risk for secondary hemochromatosis following transfusion
|
Those with beta-thalassemia
|
|
_____ is a yellowish, fat soluble pigment and the end product of lipid peroxidation
|
Lipofuscin
|
|
Combination of lipofuscin and organ atrophy is aka
|
Brown Atrophy
|
|
Metastatic calcification is caused by
|
Hypercalcemia
|
|
Often the result of
|
HyperPTH, osteolytic tumors, hypervitaminosis D
|
|
What is dystrophic calcification
|
Calcification in areas of previously damaged tissue, such as areas of old trauma, TB lesions, scarred heart valves and atherosclerotic lesions
|
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Which chaperone protein marks abnormal proteins for degradation
|
Ubiquitin
|
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Abnormal protein aggregation is characteristic of which diseases
|
Amyloidosis, Alzheimers, Prion diseases and Huntington’s
|
|
|
|
|
Inflammation
|
|
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Name the five cardinal signs
|
Red, pain, heat, swelling, LOF
|
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Name the three families of adhesion molecules
|
Selectins, integrins, Ig-family adhesion proteins
|
|
_____ are induced by IL-1 and TNF
|
Selectins
|
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L-selectins are expressed on _____ and bind to endothelial cells
|
Neutrophils
|
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E and P selectins are expressed on _____ cells and bind to leukocytes
|
Endothelial
|
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Which selectins are stored inside endothelial cells and are brought to the surface only after stimulation by histamine and/or thrombin
|
P-selectins
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______ are expressed on endothelial cells and bind to integrin molecules on leukocytes
|
ICAM-1 and ICAM-2
|
|
Give an example of the integrins expressed on leukocytes
|
LFA-1
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____ are the most prominent inflammatory cells in the first 24 hrs
|
Neutrophils
|
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Name two important causes of neutrophilia
|
Bacterial infection
|
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After 2-3 days, PMNs are replaced by
|
Monocytes/macrophages
|
|
Name some important causes of monocytosis (i.e. increased # of monocytes in peripheral blood)
|
TB, brucellosis, typhus, salmonella infection
|
|
Which are the most prominent inflammatory cells in viral infections
|
Lymphocytes (also most prominent in chronic inflammation)
|
|
Eosinophils are most prominent in
|
Allergic RXNs and parasite infections
|
|
Which cells are sources of histamine
|
Mast cells and basophils
|
|
Important causes of basophilia include
|
CML and other myeloproliferative diseases
|
|
Margination
|
Leukocytes localize to the outer margin of the blood flow
|
|
Pavementing
|
Occurs as leukocytes line the endothelial surface
|
|
Rolling (tumbling) is mediated by
|
Endothelial selectins loosely binding to leukocytes
|
|
Adhesion of leukos to endothelial surface is mediated by
|
Integrins on leukos binding to Ig-family adhesion proteins on endothelium
|
|
Transmigration across the endothelium is mediated by
|
PECAM-1
|
|
Chemotaxis?
|
Process by which leukos are attracted and move towards an injury
|
|
Movement occurs along a
|
Chemical gradient
|
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Name the most prominent chemotactic factors
|
Complement components, esp. C5a
|
|
Define opsonization
|
The coating of particulates by opsonins, which immobilize the particles on the macrophage surface
|
|
Name the most important opsonins
|
IgG subtypes
|
|
Which portion of the IgG molecule binds to the phagocytic cells
|
Fc
|
|
What is the most important intracellular microbicidal killing method
|
Oxygen dependent microbe killing
|
|
Phagocytosis initiates activity of the _____ shunt, causing an oxidative burst, which supplies electrons to NADPH oxidase in the phagosomal membrane
|
Hexose monophosphate
|
|
One of the products of the oxidase is ____, which is further converted to _____ by dismutation, which is further mutated to _____
|
Superoxide ion, hydrogen peroxide, hydroxyl radical
|
|
What does the hyd. Peroxide do for us?
|
In the presence of myeloperoxidase and a halide ion, it oxidizes microbial proteins and disrupts cell walls
|
|
Exogenous mediators of acute inflammation are most often _____ and are exemplified by _____
|
Of microbial origin, formylated peptides of E.coli
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Endogenously, _____ from mast cells, basophils and platelets ↑capillary permeability
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Histamine
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Binding of what substances to basophils/mast cells causes this acute inflammation
|
IgE, C3a / C5a (anaphylatoxins), and IL-1
|
|
The AA pathway yields ____ in platelets and _____ in endothelial cells
|
TxA2, PGI2 (prostacyclin)
|
|
Function of platelet TxA2
|
Powerful vasoconstrictor and platelet aggregant
|
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Function of endothelial PGI2
|
Powerful vasodilator and inhibitor of platelet aggregation
|
|
Function of the leukotrienes released via the lipoxygenase pathway of AA metabolism
|
Chemotactic for PMNs (LTB4)
|
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What are the acute phase responses activated by cytokines
|
Fever and leukocytosis
|
|
The kinin system is activated by
|
The Hageman factor (XIIa)
|
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Kinin converts prekallikrein to kallikrein which then cleaves HMWK to _____
|
|
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Which anaphylatoxin activates the lipoxygenase pathway of AA metabolism
|
C5a
|
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What (who) forms the MAC (membrane attack complex)
|
C5b-C9
|
|
Nitric oxide is produced by
|
Endothelial cells
|
|
Abcesses are cavities filled with pus and are usually the result of
|
Bacterial infections, esp. S.aureus
|
|
Increased susceptibility to infection may be due to deficiency of
|
Complement components C2,3,5
|
|
Describe chronic granulomatous disease of childhood
|
Usually X-linked
|
|
Which organisms are ingested but NOT destroyed
|
Catalase positive – these guys can destroy the hyd. Peroxide generated by bacterial metabolism, and the PMNs are deficient so they cannot generate additional hyd.peroxide, thus none is available as a substrate for myeloperoxidase system
|
|
Examples of CAT(+) and CAT(-) organisms
|
S.aureus – CAT(+)
|
|
What type of inheritance is Chediak-Higashi
|
Autosomal recessive
|
|
Marked by the presence of abnormal
|
WBCs
|
|
The WBCs have impaired
|
Chemotaxis and migration (microtubules)
|
|
How do they look morphologically
|
Large cytoplasmic granules (representing abnormal lysosomes)
|
|
What are the two major patterns of chronic inflammation
|
Chronic nonspecific and granulomatous inflammation
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Granulomas are nodular collections of specialized macrophages referred to as_____ and are usually surrounded by a rim of _____
|
Epitheliod cells
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Here’s the process: macrophages present antigen to _____, which in turn secrete _____ that mediate the macrophage conversion to epitheliod cells and giant cells
|
CD4+
|
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Granulomas are often characterized by _____ necrosis
|
Caseous
|
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However, non-caseating granulomatous disease is most often caused by _____
|
Sarcoidosis (think young, black female)
|
|
Which giant cells also characterize granulomas
|
Langhans giant cell
|
|
What is unique about the nuclei of these cells
|
Horseshoe nucleus
|
|
What infectious agents are classic for granulomas:
|
TB, leprosy, Blasto, Histo, Coccidio, Syphillus, Cat Scratch disease.
|
|
____ cells divide actively throughout life and are capable of regeneration after injury
|
Labile
|
|
____ cells generally undergo very few divisions but can regenerate from G-0 cells when needed. They are also capable of regenerating post-injury.
|
Stable
|
|
Examples?
|
Liver and kidney cells
|
|
What is the third class of cells and examples
|
Permanent: neurons and myocardial cells
|
|
Promotes new vessel formation?
|
|
|
_____ tissue is highly vascular, newly formed connective tissue consisting of capillaries and fibroblasts
|
Granulation
|
|
_____ is produced by fibroblasts
|
Collagen
|
|
Why does diabetes delay or impede tissue repair
|
↑susceptibility to infection and ↓circulation
|
|
Why does deficiency of ascorbic acid / protein also delay tissue repair
|
Both are required for collagen formation
|
|
|
|
|
Hemodynamic Dysfunction
|
|
|
Difference between ecchymosis and hematoma
|
Diffuse vs. local hemorrhage
|
|
Chronic passive congestion of the liver and lower extremities is most often cuased by
|
RHF
|
|
This results in nutmeg liver, explain:
|
Produced by a combination of dilated, congested central veins and the surrounding brownish-yellow (often fatty) liver cells
|
|
_____ is necrosis resulting from ischemia
|
Infarction
|
|
White or pale infarcts are usually caused by
|
Arterial occlusions in the heart, spleen or kidney
|
|
Red infarcts are
|
Hemorrhagic infarcts in which red cells ooze into the necrotic area
|
|
Red infarcts characteristically occur in
|
Lung and GI tract (i.e. areas w/redundant supplies)
|
|
What causes platelet adhesion in thrombogenesis
|
Exposed (damaged) endothelial collagen
|
|
This platelet surface glycoprotein receptor and subendothelial collagen interaction is mediated by
|
vWF
|
|
Describe the release reaction
|
Soon after adhesion, platelets release ADP, histamine, PDGF etc
|
|
Activation of platelet membrane phospholipase leads to what
|
AA pathways, thus TxA2 (recall: potent vasoconstrictor and platelet aggregant
|
|
Why don’t we become one giant clot?
|
PGI2 is also produced by endothelial cells and is an antagonist to platelet TxA2
|
|
Endothelial cells are somewhat against clotting, what substances do they secrete
|
Proteoglycans that activate AT-III
|
|
What does (APC) activated protein C do?
|
Cleaves factors Va and VIIIa
|
|
What co-factor for APC do endothelial cells synth?
|
Protein-S
|
|
Extrinsic pathway of coagulation:
|
Factor VII / Tissue Factor, Factor X/Xa etc
|
|
Factor Xa and Va activate
|
Prothrombin to thrombin
|
|
Which, in turn, activates
|
Fibrinogen to fibrin
|
|
What’s important about XIIa
|
XII-to-XIIa activation links the fibrinolytic system, coagulation system, complement system and kinin system
|
|
Hereditary thrombophilia is common in
|
Adolescents and young women
|
|
Characterized by
|
Recurrent venous thrombosis/thromboembolism
|
|
Can be caused by deficiency in
|
AT-III, protein C and/or protein S
|
|
But, what is the most common cause?
|
Factor-V Leiden – abnormal factor-V with a specific mutation that alters the cleavage site targeted by APC
|
|
This condition is aka
|
Hereditary resistance to APC
|
|
Name two more causes
|
Prothrombin 20210A transition
|
|
Which antiphospholipid antibody associated with SLE can cause a false(+) syphilis test
|
Anti-cardiolipin antibody
|
|
Mature arterial thrombi with alternating dark gray layers of platelets interspersed with lighter layers of fribrin are aka
|
Lines of Zahn
|
|
In contrast, venous clots are what color
|
Deep red
|
|
What is the most frequent form of embolism
|
Thrombo-embolism
|
|
Which type of pulmonary emboli produce sudden death
|
Saddle emboli
|
|
How do pulmonary hemorrhagic infarcts appear
|
Wedge shaped and located just below the pleura
|
|
Where do most arterial emboli develop
|
Mural thrombi in the heart
|
|
Mural thrombi in the left atrium esp/ assoc with
|
Mitral stenosis w/atrial fibrillation
|
|
Whereas, mural thrombi in the left ventricle are most often assoc. w/
|
MI
|
|
Most frequently, which cerebral artery is embolized
|
Middle cerebral artery
|
|
What are paradoxical emboli
|
Originate in venous system, but become left sided emboli
|
|
How can this happen
|
Patent foramen ovale or atrial-septal defect
|
|
What to watch out for in a pt with many fractures following trauma
|
Fat embolism syndrome
|
|
LHF results in
|
|
|
Edema can also be caused by ↓oncotic pressure. Name two causes for this:
|
Nephrotic syndrome – loss of protein, thus hypoalbuminemia
|
|
_____ aka generalized edema
|
Anasarca
|
|
Transudate has a low ____ content and SG<?
|
Protein, 1.012
|
|
Exudate results from
|
↑vascular permeability as a result of inflammation
|
|
Exudate has a high ____ content and SG>? And characteristically contains large numbers of
|
Protein, 1.020, inflammatory leukocytes
|
|
Cardiogenic shock is most often caused by
|
LHF due to MI
|
|
Septic shock most often characterized by what type of infections
|
Gram negative
|
|
What causes the cascade of cytokines that leads to direct vascular injury
|
Lipopolysaccharide (LPS) from outer membrane of gram negative bacteria
|
|
What toxic molecules are responsible for toxic shock syndrome
|
Superantigens, most often assoc. w/S.aureus
|
|
What is the most important anatomical finding in shock
|
Acute tubular necrosis
|
|
Name some others
|
Patchy mucosal hemorrhage in the colon
|
|
First Aid Path Start
|
|
|
Pt has multiple fractures, anemia, and CN deficits, what cells are dysfunctional
|
Osteoclasts (osteopetrosis)
|
|
High blood pressure in arms, low BP in legs
|
Coarctation of aorta
|
|
Pt presents with diffuse goiter and hyperthyroidism, what are the expected TSH and thyroid hormone levels
|
Low TSH, high thyroid hormone levels
|
|
Obese woman presents with hirsutism and ↑ levels of serum testosterone, what is the DX
|
Polycystic ovarian syndrome
|
|
Pt with elevated cortisol, 1 mg DEX test does not ↓cortisol, 8 mg does, what is the DX
|
Pituitary tumor
|
|
Child has been anemic since birth, splenectomy would result in ↑hematocrit in which disease
|
Spherocytosis
|
|
DX: dizziness, tinnitus, enlarged internal acoustic meatus
|
Schwannoma
|
|
DX: child with weakness and enlarged calves
|
Duchenne’s muscular dystrophy
|
|
How is it inherited
|
X-linked recessive
|
|
25 y/o female, sudden uniocular vision loss, HX of parasthesias and weakness that have resolved
|
MS
|
|
Teenager presents with nephritic syndrome and hearing loss, DX:
|
Alport’s syndrome
|
|
Female has recurrent URIs and inability to conceive, she has dextrocardia, which protein is defective
|
Dynein (Kartagener’s)
|
|
55 y/o man, heavy drinker and smoker, flu-like symptoms, no organisms on Gram stain, silver stain of sputum shows Gram(-) rods?
|
Legionella pneumonia
|
|
50 y/o man with diarrhea, his face is plethoric and a heart murmur is detected, DX:
|
Carcinoid syndrome
|
|
Elderly woman present with headache and jaw pain, labs show ↑ESR, DX:
|
Temporal arteritis
|
|
Woman at 16 weeks gestation, but has abnormally large abdomen, what could be the cause? What blood test will help with the DX?
|
Hydatidiform mole
|
|
Most likely cause of systolic crescendo-decrescendo murmur
|
Aortic stenosis
|
|
Woman of short stature with shortened 4th and 5th metacarpals, what endocrine disorder?
|
Albright’s hereditary osteodystrophy, or pseudohyoparathyroidism
|
|
30 y/o man with BRBPR after stressful event. Colonic biopsy shows transmural inflammation
|
Crohn’s Disease
|
|
Thus, what would be the key to the biopsy on UC
|
Inflammation through the mucosa/submucosa only
|
|
Young man with mental deterioration and tremors, brown ring around cornea: DX and TX
|
Wilson’s disease
|
|
Gross photo of AAA, what is the most likely cause
|
Atherosclerosis
|
|
Photo of bunch-of-grapes hydatidiform mole, high levels of what substance are found
|
hCG
|
|
Photo of focal intestinal hemorrhages in a weightlifter, DX
|
Strangulation of a hernia
|
|
Collapse of middle lobe of right lung, pt has a mass In right bronchus, HX of recurrent pneumonia
|
Bronchogenic carcinoma
|
|
Middle aged female w/syncope, mass removed from right atrium, wispy mucus-like tissue
|
Myxoma
|
|
What are the clinical findings in pneumothorax
|
Pleuritic pain, SOB
|
|
1 y/o baby with red spot on face, it blanches w/pressure, no neurological signs, what is the expected course
|
Regression
|
|
What similar stain has a much more malignant DX
|
Port-wine stain of Sturge-Weber syndrome
|
|
H&E of lung biopsy from a plumber shows dumb-bell shaped stuctures, DX?
|
Asbestosis
|
|
Increased risk for?
|
Malignant mesothelioma
|
|
Multiple mesangial nodules on a glomerulus is indicative of
|
Diabetes mellitus
|
|
Polycystic kidney disease in adult is inherited by
|
Auto. Dominant
|
|
Pt with anemia, hypercalcemia, and bony pain on palpation; bone marrow biopsy shows many plasma cells w/large, round, off-center nucleus
|
Multiple myeloma
|
|
NTDs are asscoiated with ↑levels of
|
Alpha-feto protein in both amniotic fluid and in mother’s serum
|
|
1.
|
early cyanosis (blue babies)
|
|
2.
|
late cyanosis (blue kids)
|
|
1.
|
R-to-L
|
|
2.
|
L-to-R
|
|
What are the three T’s of blue babies
|
1.
|
|
2.
|
Transposition of great vessels
|
|
3.
|
Truncus arteriosus
|
|
Children may squat to ↑
|
Venous return
|
|
What three conditions lead to blue kids
|
VSD > ASD > PDA
|
|
In fact, ___ is the most common congenital anomaly
|
VSD
|
|
Progressive pulmonary HT leads to _____ shunt, aka _____
|
R-to-L, Eisenmenger’s
|
|
Describe Eisenmenger’s syndrome
|
Uncorrected VSD, ASD or PDA leads to progressive pulm. HT, the shunt changes from L-to-R over to R-to-L, which causes late cyanosis, clubbing and polycythemia
|
|
PROVe pneumonic of Tetralogy of Fallot
|
Pulmonary stenosis
|
|
Patients suffer _____ spells
|
Cyanotic
|
|
Cause of the tetralogy?
|
Anterosuperior displacement of the infundibular septum
|
|
Is transposition of the great vessels compatible with life
|
No, not unless a shunt is present to allow mixing of blood. VSD, PDA or PFO
|
|
Which babies are at high risk
|
Those born to diabetic mothers
|
|
In coarctation of aorta, what is the infantile type and adult type
|
Infantile: stenosis proximal to insertion of ductus arteriosus (preductal)
|
|
Gender bias?
|
Male:female = 3:1
|
|
Any associated syndromes
|
Infantile is assoc. w/Turner’s syndrome
|
|
Fetal PDA is a ‘normal’ R-to-L shunt, but perinatal, is the PDA remains, flow switches and RHF results, what type of murmur is heard
|
|
|
What maintains patency
|
PGE synthesis, Low oxygen tension
|
|
Down’ syndrome risk is
|
1:700
|
|
Duodenal _____ is common, as is congenital _____
|
Atresia, heart disease
|
|
1.
|
which leukemia?
|
|
2.
|
Which neurological deterioration
|
|
1.
|
ALL
|
|
2.
|
ALLzheimer’s
|
|
95% of cases are due to
|
Meiotic non-disjunction (MNDJ) of homologous chromosomes
|
|
MNDJ is assoc. with
|
↑age
|
|
Edward’s syndrome is trisomy ____
|
18 (Election age is 18)
|
|
Patau’s syndrome is trisomy ____
|
13 (Puberty is at 13)
|
|
Which is assoc. with midline defects
|
Patau’s
|
|
Which is asoc. with polydactyly
|
Patau’s
|
|
What is one of the most common causes of hypo-gonadism in males
|
Klinefelter’s
|
|
Genotype
|
XXY
|
|
Presence of inactivated X chromosome (aka____)
|
Barr body
|
|
Whereas, in Turner’s syndrome, there is____
|
No Barr body present, because genotype = XO
|
|
Turner’s is most common cause of
|
Primary amenorrhea
|
|
Cardiac anomalies?
|
Coarctation of the aorta
|
|
Double ‘Y’ males (XYY) are phenotypically normal except for
|
Very tall, severe acne
|
|
Define pseudo-hermaphrodite
|
Disagreement between external genitalia and testes/ovaries (i.e. phenotypic vs. gonadal sex), ambiguous genitalia
|
|
Most common form of male pseudo- is
|
Testicular feminization (androgen insensitivity),
|
|
This results from a mutation in
|
The androgen receptor gene
|
|
Define a true hermaphrodite
|
Both ovary and testicular tissue present
|
|
microcephaly, retadation, high pitched crying
|
|
|
X-linked defect affecting the methylation and expression of FMR-1 gene. It is the 2nd most common cause of mental retardation
|
Fragile X syndrome
|
|
____ repeat disorder (CGG)
|
Triple
|
|
Phenotype (appearance)
|
Enlarged testes, long face, large jaw, autism
|
|
What is the MOST common cause of retardation
|
Downs
|
|
1. X-linked recessive, dystrophin gene deletion, onset <5y/o, starts in pelvic girdle, big calves
|
|
|
Why big calves (pseudohypertrophy)
|
Fatty-fibrous replacement of muscle
|
|
Gower’s maneuver
|
Using arms to stand one’s self up (indicates proximal limb weakness)
|
|
How does Becker’s MD differ
|
Dystrophin gene mutations, NOT deletions, thus not as severe
|
|
How is diagnosis made
|
Muscle biopsy, serum CPK increase
|
|
CF is due to
|
CFTR gene defect (auto. Recessive) on C7
|
|
Recurrent pneumonia often due to which organisms
|
Pseudomonas, S. aureus
|
|
How does this manifest itself in newborns
|
Meconium ileus, failure to thrive
|
|
How about in adults (esp. males)
|
Infertility, vitamin ADEK deficiencies
|
|
|
|
|
Autosomal dominant diseases (8)
|
|
|
Adult polycystic kidney disease: 90% of cases due to mutation in
|
APKD1 gene on C16
|
|
Associated conditions
|
Berry aneurysms, mitral valve prolapse
|
|
Isn’t there a juvenile form as well?
|
Yes – but its recessive
|
|
_____: elevated LDL due to defective LDL receptor
|
Familial hypercholesterolemia
|
|
Look for severe _____ early in life
|
Atherosclerotic disease, cholesterol 300-700
|
|
Classic finding is xanthomas on
|
The Archille’s tendon
|
|
Marfan’s is due to
|
Fibrillin gene mutation
|
|
Thus, characterized by
|
Connective tissue disorders
|
|
Long thin skeletons, fingers etc. But, what cardiac anomalies are present
|
Cystic medial necrosis of aorta, dissecting aortic aneurysms, floppy mitral valve (prolapsing)
|
|
Any ocular findings
|
Sublaxation of lens
|
|
_____: café au lait spots, neural tumors, Lisch nodules (pigmented iris hamartomas)
|
NFT1: von Recklinghausens (chromosome 17 because there are 17 letters in name)
|
|
Findings in von Hippel Lindau
|
Hemangioblastomas of retina / cerebellum / medulla, about 50% get renal carcinomas
|
|
Which gene is involved
|
VHL gene on C3
|
|
_____ disease: depression, progressive dementia, chorea
|
Huntington’s
|
|
What transmitters are affected
|
↓GABA and Ach in the brain
|
|
Section of brain most affected
|
Caudate nucleus
|
|
How is this like Fragile X syndrome
|
Triple repeat disorder
|
|
Which chromosome is involved
|
C4
|
|
Name the features of Familial Adenomatous Polyposis
|
Deletion on chromosome Five
|
|
_____ causes hemolytic anemia and splenectomy is curative
|
Hereditary spherocytosis
|
|
How is MCHC affected
|
Increased
|
|
End of autosomal dominant disease
|
|
|
At this point, you should study the lists of auto.recessive and x-linked diseases
|
|
|
|
|
|
Why are female carriers of x-linked diseases rarely affected?
|
B/c of random inactivation of X chromosomes in each cell.
|
|
___ are assoc. W/low folic acid during pregnancy
|
NTDs
|
|
Lab tests?
|
Raised alpha-fetoprotein
|
|
What are the defining features of spina bifida occulta
|
Failure of boney spinal canal closure
|
|
Meningocele?
|
Meninges alone herniated through spinal canal defect
|
|
Meningomyelocele, therefore, is when
|
Meninges AND spinal cord herniated through
|
|
Even though there is no herniation in occulta, what telltale sign is present
|
Tuft of hair on child’s back
|
|
When is the highest risk (during pregnancy) for FAS (fetal alcohol syndrome)
|
3-8 weeks
|
|
What is the number one cause of congenital malformations in the US?
|
FAS
|
|
____ is the abnormal proliferation of cells with loss of size, shape and orientation
|
Dysplasia
|
|
What two conditions define carcinoma-in-situ
|
1. Malignant cells have not invaded basement membrane
|
|
How do malig. Cells breach the basement membrane
|
Using hydrolases and collagenases
|
|
When the BM is breached, this is called
|
Invasive carcinoma
|
|
In the “seed and soil” theory of metastases, the tumor embolus is the seed, what is the soil
|
The target organ (liver, lungs, bone, brain)
|
|
When one adult cell type is replaced by another, we call this
|
Metaplasia
|
|
_____ = abnormal cells lacking differentiation
|
Anaplasia
|
|
_____ = a clonal proliferation of cells that is uncontrolled and excessive
|
Neoplasia
|
|
The histiologic appearance of a tumor is aka
|
Grade (usually I-IV)
|
|
Grading is based on degree of differentiation and number of
|
Mitoses per HPF
|
|
The STAGE is based on
|
Site and size of primary lesion, spread to LNs, and presence of mets.
|
|
Which has more prognostic value
|
Stage
|
|
Describe the TNM staging system
|
T=size of tumor
|
|
5. bone
|
|
|
Down’s
|
|
|
Xeroderma pigmentosum
|
Squamous cell and BCC of skin
|
|
Chronic atrophic gastritis, pernicious anemia
|
Gastric adenocarcinoma
|
|
Tuberous sclerosis (facial angiofibroma, seizures, mental retardation)
|
Astrocytoma and cardiac rhabdomyoma
|
|
Actinic keratosis
|
SCC of skin
|
|
Barret’s
|
Esophageal adenocarcinoma
|
|
Plummer Vinson syndrome
|
SCC of esophagus
|
|
What are the symptoms of PV syndrome
|
Atrophic glossitis, esophageal webs, anemia
|
|
These are all due to
|
Iron deficiency
|
|
Cirrhosis leads to (ETOH, Hep B or C)
|
Hepatocellular carcinoma
|
|
UC leads to
|
Colonic adenocarcinoma
|
|
Paget’s disease of bone
|
Secondary osteosarcoma and fibrosarcoma
|
|
Immunodeficiency states most often lead to
|
Malignant lymphomas
|
|
Autoimmune diseases such as Hashimoto’s and MG can lead to
|
Malignant thymomas
|
|
Acanthosis nigricans (hyperpigmentation and epidermal thickening
|
Visceral malignancy (stomach, lung, breast, uterus)
|
|
Dysplastic nevus leads to
|
Malignant melanoma
|
|
|
|
|
Given an oncogene, name the assoc. tumor:
|
|
|
c-myc
|
Burkitt’s lymphoma
|
|
Bcl-2
|
Follicular and undifferentiated lymphomas
|
|
Erb-B2
|
Breast, ovarian, gastric carcinomas
|
|
Ras
|
Colon carcinoma
|
|
|
|
|
Name the chromosomes the following genes are located on, and the tumor that results with their loss
|
|
|
Rb
|
13q – retinoblastoma, osteosarcoma
|
|
BRCA ½
|
13q,17q – breast and ovarian cancer
|
|
P53
|
17p – most human cancers
|
|
|
|
|
PSA stands for
|
Prostatic acid phosphatase
|
|
Which marker is very non-specific but is produced by 70% of colorectal and pancreatic cancers
|
CEA (carcinoembryonic antigen)
|
|
Which carcinoma elevates alpha-fetoprotein
|
Hepatic carcinoma
|
|
Beta –hCG is a good marker for
|
Hydatidiform moles, Choriocarcinomas, Gestational trophoblastic tumors
|
|
S-100 is a good marker for
|
Melanoma, neural tumors, and astrocytomas
|
|
Alk-phosphatase is elevated in
|
Paget’s disease of bone
|
|
|
|
|
Name the assoc. disease/cancer (given the virus)
|
|
|
HTLV-1
|
Adult T-call leukemia
|
|
HBV,HCV
|
Hepatocellular carcinoma
|
|
EBV
|
Burkitt’s, nasopharyngeal carcinoma
|
|
HPV
|
Cervical carcinoma (16,18)
|
|
HHV-8
|
Kaposi’s sarcoma
|
|
|
|
|
Given a toxin, name the affected organ
|
|
|
Aflatoxin, vinyl chloride
|
Liver
|
|
Nitrosamines
|
Esophagus, stomach
|
|
Asbestos
|
Lung (mesothelioma, bronchogenic carcinoma)
|
|
Arsenic
|
Skin (SCC)
|
|
CCl4
|
Centrilobar necrosis, fatty change
|
|
|
|
|
|
|
|
Prostate adenocarcinoma most often arises from which lobe
|
Posterior lobe (peripheral zone)
|
|
_____ is a presursor to SCC
|
Actinic keratosis
|
|
SCC is assoc. with what exposures
|
Sunlight and arsenic
|
|
Locally invasive but rarely
|
Metastasize
|
|
Histology?
|
Keratin pearls
|
|
What is the histology of BCC
|
Palisading nuclei
|
|
What about the pathognomonic gross appearance
|
Pearly papules
|
|
Any metastases
|
Almost never
|
|
So, who DOES metastasize
|
Melanoma
|
|
____ of tumor correlates with risk of mets
|
Depth
|
|
____ is a precursor
|
Dysplastic nevus
|
|
Common benign bone tumor in men <25y/o
|
Osteochondroma
|
|
Commonly originates from
|
Long metaphysis
|
|
Another common benign bone tumor that occurs at epiphyseal end of long bones, onset between 20-40 y/o. (distal femur, proximal tibia)
|
Giant cell tumor
|
|
Characteristic appearance on xray?
|
Soap bubble
|
|
Most common primary malig. Tumor of bone in men 10-20 y/o.
|
Osteosarcoma
|
|
Location?
|
Metaphysis of long bones
|
|
Any predisposing factors?
|
Paget’s disease, familial retinoblastoma
|
|
Characteristics on xrays?
|
Codman’s triangle
|
|
Another malig. Sarcoma in boys < 15 y/o?
|
Ewing’s sarcoma
|
|
Xray characteristics?
|
Onion-skin pattern
|
|
What type of cells are involved
|
Anaplastic SMALL cells
|
|
Location in bone?
|
Diaphysis of long bones, pelvis, scapula, ribs
|
|
Which translocation?
|
11;22 translocation
|
|
|
|
|
Brain tumors
|
|
|
Adult tumors are mostly located______
|
Supratentorial (kids are infratentorial)
|
|
GBM is most common primary brain tumor. What is its location and PX
|
Cerebral hemispheres
|
|
Histology
|
Pseudopalisading tumor cells border areas of necrosis and hemorrhage
|
|
What’s the second most common
|
Meningiomas
|
|
Locations?
|
Convexities of hemispheres
|
|
Arise from _____ cells
|
Arachnoid
|
|
Histology
|
Psammoma bodies (whorled pattern)
|
|
What rare tumors grow in the frontal lobe and have fried egg appearance
|
Oligodendrogliomas
|
|
Name the third most common tumor
|
Scwannomas
|
|
Often localized to ___ nerve and called
|
8th, acoustic neuroma
|
|
Bilateral schwannomas found in
|
NF2 syndrome
|
|
Which adenoma is derived from Rathke’s pouch
|
Pituitary
|
|
Symptoms
|
Prolactin increases
|
|
Now, for some childhood brain tumors
|
|
|
Highly malignant cerebellar tumor that is a form of primitive neurectodermal tumor (PNET)
|
Medulloblastoma
|
|
Symptoms
|
Can compress 4th ventricle and cause hydrocephaly
|
|
Histiology
|
Rosette or perivascular perirosette
|
|
What other cerebellar tumor is asoc. With von Hippel Lindau syndrome when seen with retinoblastomas
|
Hemangioblastomas
|
|
These tumors can produce ____ leading to ____
|
EPO, polycythemia
|
|
Which tumors are found most often in the 4th ventricle
|
Ependyomas
|
|
Which tumor are most often found in the posterior fossa
|
Low-grade astrocytomas
|
|
Childhood tumor derived from Rathke’s pouch that mimics pituitary adenoma b/c it can also cause bilateral hemianopia
|
Craniopharyngioma
|
|
_____ is common, which helps with radiologic DX
|
Calcification
|
|
|
|
|
Lots of Bad Stuff Kills Glia
|
Lung
|
|
What are the most common sites of mets (after LNs)
|
Liver and Lung
|
|
Cancer Sometimes Penetrates Benign Liver
|
Colon>Stomach>Pancreas>Breast>Lung
|
|
BLT with a Kosher Pickle
|
Breast, Lung, Thyroid, Testes, Kidney, Prostate
|
|
What is the MOST common organ receiving mets
|
Adrenals (due to rich blood supply), first in medulla, then spreads to the rest of the gland
|
|
Most common organ to SEND mets?
|
Lung > breast & stomach
|
|
|
|
|
Name the paraneoplastic effect of the tumor:
|
|
|
Small cell of lung
|
ACTH (Cushing’s)
|
|
Small cell of lung and intracranial neoplasms
|
ADH (leads to SIADH)
|
|
Squamous cell of lung
|
PTH-related peptide (leads to hypercalcemia)
|
|
Name some more tumors with this same effect
|
Renal cell, breast cancer, multiple myeloma
|
|
Renal cell carcinoma
|
EPO (leads to polycythemia)
|
|
Thymoma / bronchogenic carcinoma
|
Antibodies against presynaptic calcium channels at NMJ
|
|
What can this lead to?
|
Lambert-Eaton syndrome
|
|
What cause microcytic, hypochromic anemia (MCV<80)
|
Iron deficiency
|
|
Lab results
|
TIBC↑, ferritin↓, serum iron↓↓
|
|
What else can cause this type of anemia
|
Thallasemia and lead poisoning
|
|
Note that vit B12 and folate deficiencies are both associated with
|
Hypersegmented PMNs
|
|
What causes macrocytic (MCV>100) anemia
|
B12 and folate deficiencies
|
|
What else?
|
Drugs that block DNA synthesis
|
|
Examples?
|
Sulfa, trimethoprim, AZT
|
|
Normocytic, normochromic anemia causes:
|
|
|
Enzyme defects
|
G6PD, PK deficiency
|
|
RBC membrane defects
|
Hereditary spherocytosis
|
|
Bone marrow disorders
|
Aplastic anemia, leukemia
|
|
Hemoglobinopathies
|
Sickle cell
|
|
Autoimmune pblms
|
Hemolytic anemia
|
|
Common battlefield cause???
|
Hemorrhage
|
|
How do the following labs change with anemia of chronic disease: TIBC, ferritin, iron
|
TIBC↓, ferritin↑, serum iron↓, ↑storage iron in marrow macrophages
|
|
↓serum haptoglobin and ↑serum LDH indicate
|
RBC hemolysis
|
|
How do we distinguish between immune mediated and non-immune mediated hemolysis
|
Coombs Test
|
|
TIBC, serum iron
|
TIBC = normal
|
|
_____ anemia: pancytopenia, severe anemia, failure or destruction of multipotent myeloid stem cell lines
|
Aplastic
|
|
Causes?
|
Radiation, benzene, chloramphenicol, some viruses
|
|
What are the pathological features (ir, cell morphology, marrow presentation)
|
Pancytopenia with normal cell morphology
|
|
Characterize hereditary spherocytosis
|
Intrinsic, extravascular hemolysis due to spectrin defect
|
|
How do RBCs appear
|
Small, round, no central pallor
|
|
Lab tests to confirm?
|
HgB↑ (MCV↑) b/c there is ‘less’ membrane
|
|
How to distinguish from Warm Antibody Hemolysis?
|
Use a Coombs test. WAH is (+), hereditary spherocytosis is Coombs (-)
|
|
|
|
|
Blood dyscrasias
|
|
|
What causes sickle cell
|
Single amino acid substitution in the beta-HgB chain: substitute glutamic acid with valine
|
|
What leads to sickling
|
Low oxygen environment, dehydration
|
|
How do heterozygotes (trait carriers) benefit
|
Malaria resistant
|
|
What diseases are homozygotes at ↑risk for
|
Aplastic crisis
|
|
What TX for HbS involves ↑HbF
|
Hydroxyurea
|
|
Alpha-thalassemia is due to missing alpha-globins (up to 4). If all 4 are missing, what results in utero
|
Hydrops fetalis and death
|
|
Beat-minor vs. Beta-major thallassemia
|
Minor=beta chain underproduced (heter)
|
|
Thalassemia is prevalent in ____ populations
|
Mediterranean
|
|
Beta-thal. Major can result in
|
Cardiac failure due to secondary hemochromatosis
|
|
What is the MOST common cause of DIC
|
Obstetric causes (amniotic embolus)
|
|
Name three more common causes
|
Gram(-) sepsis, transfusion, trauma, malignancy
|
|
Lab findings
|
PT↑ PTT↑ Fibrin split ptoducts↑ platelets↓
|
|
What normaly brings in a pt with platelets pblms
|
Gums bleed
|
|
Whereas, coagulation factor defects bring in the pt with
|
Hemarthroses, bruising, macrohemorrhage
|
|
Causes of platelet pblms include
|
ITP, TTP, DIC
|
|
Hemophilia A =
|
Factor VIII deficiency
|
|
Hemophilia B =
|
Factor IX deficiency
|
|
What is the most common bleeding disorder
|
Von Willebrand’s
|
|
Multiple myeloma: monoclonal ____ cell with fried-egg appearance. Produces large amounts of which Ig?
|
Plasma call
|
|
Pt presents with
|
Bone lesions and hypercalcemia, ↑susceptibility to infection and anemia
|
|
Xray findings?
|
Punched out lesions
|
|
Associated with primary _____
|
amyloidosis
|
|
What is characteristic on serum protein electrophoresis
|
“M” spike
|
|
Urinalysis?
|
Ig light chains (Bence Jones proteins)
|
|
Blood smear show anything?
|
RBCs stacked like poker chips (rouleau formation)
|
|
How does MM compare with Waldenstroms macroglobinemia?
|
The “M” spike is IgM and there are NO lytic lesions
|
|
What causes achalasia
|
Loss of myenteric plexus (Auerbachs) results in failure of relaxation of LES
|
|
Assoc. w/↑risk of
|
Esophageal carcinoma
|
|
Secondary achalasia results from
|
Chaga’s disease
|
|
Barium swallow shows
|
Bird’s beak
|
|
Barrett’s: replacement of ___ with ___
|
Stratified squamous, columnar
|
|
Barrett’s becomes _____
|
Adenocarcinoma
|
|
2. Type B
|
|
|
Both types ↑ risk of
|
Gastric carcinoma
|
|
Name the 2 types of PUD
|
Gastric and duodenal
|
|
Which has ↑ pain w/meals and weight loss
|
Gastric
|
|
Compare H. pylori infection in the two types
|
70% in gastric
|
|
How does food affect duodenal ulcers
|
Pain is relieved, thus, weight gain
|
|
Duodenal ulcers due to
|
1. ↑ gastric acid secretion
|
|
Duodenal also assoc. with hypertrophy of
|
Brunner’s glands
|
|
Who has 2X risk of PUD
|
Smokers
|
|
|
|
|
Chrohn’s vs. UC
|
|
|
Which is infectious and which is autoimmune
|
Crohn’s: infectious, UC: autoimmune
|
|
Location affected?
|
Crohn’s: anywhere in GI tract / SKIP lesions
|
|
Who is transmural
|
Crohn’s
|
|
Which has cobblestone appearance
|
Crohn’s
|
|
Bowel wall thickening (string-sign on xray)
|
Crohn’s
|
|
Which has mucosal pseudopolyps and freely hanging mesentery
|
UC
|
|
Crypt abscesses and ulcers
|
UC
|
|
Which leads to colorectal carcinoma
|
UC
|
|
Which causes malabsorption deficiencies
|
Crohn’s
|
|
Which leads to toxic megacolon
|
UC
|
|
Creeping fat????
|
Crohn’s
|
|
|
|
|
Risk factors for diverticulosis
|
Age (>60 50%), low-fiber diet
|
|
Most common location in bowel
|
Sigmoid colon
|
|
Classic presenting symptoms
|
LLQ pain
|
|
Hirschsprung’s disease: absence of
|
Parasympathetic ganglion cells (Auerbach’s and Meissner’s plexuses)
|
|
Due to failure of
|
Neural crest migration
|
|
The dilated portion of colon is _____ to the aganglionic segment
|
Proximal
|
|
Is Putz-Jeghers a risk factor for colon cancer?
|
No
|
|
Diffuse fibrosis of liver with nodular regeneration=
|
Cirrhosis
|
|
What do the nodules look like
|
Micronodular (<3mm in size)
|
|
When would the nodules be macro-?
|
Significant liver injury leading to necrosis (post-infectious or drug induced)
|
|
In alcoholic hepatitis, AST:ALT ratio >?
|
Usually greater than 1.5
|
|
Budd-Chiari syndrome: associated with?
|
PCV, pregnancy, hepatocellular carcinoma
|
|
What exactly happens in BCS?
|
IVC occlusion (or hepatic veins) leads to congestive liver disease
|
|
_____ is failure of copper to enter circulation in the form of ceruloplasmin
|
Wilson’s disease
|
|
Neurologic side effects
|
Dementia, choreiform mvmts, basal ganglia degen.
|
|
“Classic” finding
|
Keyser-fleischer rings
|
|
Absent UDP-glucuronyl transferase, early death
|
Crigler-Najjar
|
|
Findings?
|
Jaundice, kernicterus, ↑unconj. Billirubin
|
|
What is kernicterus?
|
Billirubin deposited in brain
|
|
TX
|
Plasma phoresis / phototherapy
|
|
What is the milder form?
|
Gilbert’s
|
|
What condition causes ↑conjugated bilirubin due to defective liver excretion
|
Dubin-Johnson syndrome
|
|
Gross pathology?
|
Black liver
|
|
What is the most common primary malignant tumor in the adult liver
|
Hepatocellular carcinoma (aka hepatoma)
|
|
↑incidence assoc. with
|
Hep B/C, Wilson’s, hemochromatosis, alpha1-anti-trypsin deficiency, ETOH cirrhosis, aflatoxin B1
|
|
What two carcinomas are commonly spread by hematogenous dissemination
|
Renal and Hepatocellular
|
|
Child with hepatomegaly, coma and hypoglycemia, what could be the cause
|
Reye’s syndrome
|
|
Which viruses are involved
|
VZV, Influenza B
|
|
Name the three types of gallstones
|
Cholesterol, pigmented, mixed
|
|
|
|
|
Which is most common
|
Mixed
|
|
Which do Native Americans get
|
Cholesterol
|
|
What type do alcolholic get
|
Pigmented
|
|
What are the Four risk factors for stones
|
Fat, Fertile, Female over Forty
|
|
What is Charcot’s Triad
|
Epigastric/RUQ pain
|
|
Epigastric abdominal pain radiating to back is probably
|
Acute pancreatitis
|
|
Likely labs
|
↑amylase and ↑lipase
|
|
Lots of causes for acute, but chronic pancreatitis is strongly assoc. with
|
Alcoholism
|
|
Tumors (adenocarcinoma) in the pancreas are most often in the
|
Head (obstructive jaundice)
|
|
What is Trousseau’s syndrome
|
Migratory thrombophlebitis
|
|
|
|
|
Lungs
|
|
|
What is the hallmark of obstructive lung disease
|
FEV/FVC ratio is decreased
|
|
Why
|
Air gets trapped inside the lungs
|
|
Who are the blue-bloaters
|
Those with chronic bronchitis
|
|
DX
|
Productive cough for >3 consecutive months in 2 or more years
|
|
Hypertrophy of the mucous secreting glands in the bronchioles leads to a Reid index >?
|
50%
|
|
Leading cause?
|
Smoking
|
|
Who are the Pink Puffers
|
Emphysema pts
|
|
Enlargement of___ with decreased ___
|
Air spaces, recoil
|
|
If caused by smoking, it is
|
Centriacinar
|
|
If caused by alpha1-antitrypsin deficiency, it is
|
Panacinar
|
|
In panacinar, you would also look for
|
Liver cirrhosis
|
|
PE findings?
|
↓breath sounds, tachycardia
|
|
Which two lung conditions cause ↓I/E ratios
|
Asthma and emphysema
|
|
____ is a chronic necrotizing infection of bronchi
|
Bronchiectasis
|
|
How do the cilia play a role
|
Poor mvmt, ↑infections causes purulent sputum
|
|
Restrictive lung diseases characterized by
|
Restricted expansion, thus, ↓lung volumes (↓VC and ↓TLC)
|
|
What do the PFT’s look like?
|
FEV/FVC > 80%
|
|
Name two diseases that can cause poor muscular effort
|
Polio
|
|
How do pneumonia and pulm. Edema lead to RLD
|
Defective alveolar filling
|
|
Interstitial fibrosis causes increased recoil which is the same as saying
|
Decreased compliance
|
|
Lethal complications?
|
Cor pulmonale
|
|
Can be due to pulomany fibrosis caused by which two drugs
|
Bleomycin
|
|
obstructive
|
|
|
Asbestosis has two histological features
|
Ferruginous bodies (fiber coated w/hemosiderin)
|
|
Surfactant is produced after the ___ week
|
35th
|
|
What ratio is a measure of lung maturity
|
Lecithin-to-sphingomyelin in the amniotic fluid
|
|
What is this ratio in neonatal RDS
|
<1.5
|
|
Chemical name for surfactant
|
Dipalmitoyl phosphatydlcholine
|
|
TX
|
Maternal steroids pre-birth, surfactant at birth
|
|
What is Kartagener’s syndrome
|
Immotile cilia due to defective dynein arm
|
|
How does this affect fertility (male) and lung function
|
Sterility
|
|
What other MAJOR defect is seen
|
Situs inversus
|
|
Which lung cancer has a clear link to smoking
|
Squamous and small cell
|
|
Which tumor arise peripherally in the lung
|
Adenocarcinoma
|
|
Which is thought to NOT be related to smoking
|
Bronchioalveolar
|
|
Which organism causes lobar pneumonia
|
Pneumococcus
|
|
Characteristics
|
Intra-alveolar exudates / consolidation of up to entire lung
|
|
What organisms cause bronchopneumonia
|
S. aureus, H. flu, Klebsiella, S. pyogenes
|
|
Looks like
|
Acute inflammatory infiltrates from bronchioles into alveoli
|
|
Whereas, interstitial pneumonia looks like
|
Inflammation localized to interstitial areas at alveolar walls
|
|
And its common bugs are
|
RSV, adenoviruses, mycoplasma, legionella
|
|
Pancoast’s tumor characteristics
|
Ptosis, miosis, anhydrosis
|
|
What plexus is involved
|
Cercical sympathetic
|
|
|
|
|
What is the most common cause of dementia in elderly
|
Alzheimer’s
|
|
Senile plaque have the
|
Amyloid core
|
|
What has the abnormally phosphorylated tau protein
|
Neurofibrillary tangles
|
|
Familial form of AD associated with chromosomes
|
1,14,19,21
|
|
Which disease is specific for the frontal / temporal lobes and has intracytoplasmic inclusion bodies
|
Pick’s
|
|
Lewy bodies, substantia nigra, MPTP in street drugs….
|
Parkinson’s
|
|
2 spinocerebellar diseases
|
Olivopontocerebellar atrophy
|
|
Baby born floppy and with tongue fasciculations??
|
Werndig-Hoffman disease (motor neurons)
|
|
Disease with only lower-motor neuron symptoms
|
Polio
|
|
Necrosis, hemorrhage, ‘butterfly’ distribution with poor PX
|
GBM (adults)
|
|
What order doe these tumors occur in children: medulloblastoma, astrocytoma, ependymoma
|
A > M > E
|
|
Which hematoma results from ruptured middle meningeal artery
|
Epidural (lucid interval and death)
|
|
Subdural caused by
|
Bridging veins – seen in infants/elderly
|
|
|
|
|
Subarrachnoid hemorrhage usually do to
|
Berry aneurysm (or AVM)
|
|
What’s seen on LP
|
Blood
|
|
Parenchymal brain hematomas due to
|
HT, amyloid angiopathy, DM
|
|
Where do berry aneurysms occur
|
Bifurcation in the circle of willis
|
|
Most commonly at the
|
Ant. Communicating artery
|
|
Berry aneurysms most often assoc. with
|
ADPKD
|
|
What’s seen in the CSF of MS patients
|
↑protein (IgG)
|
|
Classic triad of MS: SIN
|
Scanning speech
|
|
What is PML
|
Progressive multifocal leukoencephalopathy
|
|
Inflammation and demyelination of peripheral nerves and motor fibers of ventral roots
|
|
|
Pt presents with
|
Symmetric ascending muscle weakness beginning in lower distal extremities
|
|
Is GB assoc. with any particular infections
|
HSV, C. jejuni, inoculations and stress
|
|
Polio is transmitted via
|
Fecal-oral
|
|
Lifecycle?
|
Replicates in oropharynx and small intestine before spreading to blood / CNS
|
|
Once in the CNS, it destroys
|
Anterior horn of the spinal cord, leading, in turn, to LMN destruction
|
|
Virus can be recovered from
|
Stool or throat
|
|
|
|
|
Most common cause of adult seizures
|
Tumor, trauma, stroke
|
|
And kids?
|
Genetic, infection, trauma
|
|
Name the partial seizures
|
Simple partial
|
|
Name the generalized seizures
|
Absence – blank stare (petit mal)
|
|
Most common cause of seizures in the elderly
|
Stroke, tumor, trauma
|
|
Wernicke’s
|
|
|
Superior temporal gyrus=
|
Broca’s area
|
|
Horner’s involves a lesion above what level
|
T1
|
|
What pathway is interrupted in Horner’s
|
Oculosympathetic pathway
|
|
______ : a softening and cavitation around central canal of spinal cord
|
Syringomyelia
|
|
How would pt present
|
Bilateral loss of pain and temp sensation in upper extremeties w/o loss of touch
|
|
Level or injury
|
C8-T1
|
|
Fibers of which tract are damaged
|
Spinothalamic
|
|
Degeneration of dorsal columns and roots due to tertiary syphilis, resulting in impaired proprioception and locomotor ataxia
|
Tabes dorsalis
|
|
Other symptoms/signs
|
Shotting (lightening) pain
|
|
What are the systemic features of RA
|
Fever, fatigue, pleuritis, pericarditis
|
|
What type of Ig’s are the RA antibodies
|
Anti-IgG antibodies
|
|
Gout: precipitation of ____ into joints due to ____
|
Monosodium urate crystals, hyperuricemia
|
|
Cuased by
|
Lesch-Nyhan, PRPP excess, glucose-6-phospatase deficiency or just decreased excretion
|
|
This last category can be due to which RX
|
Thiazides (competitive inhibitors)
|
|
Pdagra?
|
MTP joint of big toe
|
|
Symmetric or asymmetric involvement?
|
Asymmetric
|
|
What is pseudogout caused by
|
Calcium pyrophosphate crystals
|
|
Shape of crystal
|
GOUT: needle
|
|
Classic joint involvement
|
Knee (large joints in general)
|
|
Gender bias
|
GOUT: men
|
|
TX for P-GOUT?
|
None
|
|
Findings in celiac sprue
|
Northern Europeans
|
|
SLE causes LSE?
|
Libman-Sacks Endocarditis
|
|
SLE can cause false (+) _____ tests
|
Syphilis
|
|
What is LSE?
|
Non-bacterial verrucous vegetations on valves
|
|
SLE is a risk for _____ phenomenon
|
Raynaud’s
|
|
What kind of lesions seen in kidney
|
Wire-loop
|
|
Labs to DX
|
ANA (sensitive, but NOT specific to SLE)
|
|
SLE is most common and severe in what population
|
Black, female
|
|
What drugs responsible for reversible SLE syndrome
|
INH
|
|
What disease is prevelant in black females and is known for widespread non-caseating granulomas
|
Sarcoidosis
|
|
What enzyme is elevated
|
ACE
|
|
GRAIN for sarcoidosis
|
Gammaglobulinemia
|
|
HLA-B27 most often associated with
|
Ankylosing spondylitis
|
|
Which is a sero-negative spondyloarthropathy
|
Reiter’s
|
|
Classic triad
|
Can’t see, pee, or dance
|
|
Dry mouth, dry eyes and an arthritis?
|
Sjoergen’s
|
|
What gland is enlarged in Sjoergen’s
|
Parotid
|
|
Predominantly affects _____ between ___and ___
|
Females, 40-60
|
|
Dry eyes, mouth, nose and vagina along with chronic bronchitis and reflux esophagitis=?
|
Sicca syndrome
|
|
_____ = excessive fibrosis and collagen deposition throughout the body
|
Scleroderma
|
|
Diffuse scleroderma assoc. with _____ antibody
|
Anti-Scl-70
|
|
CREST syndrome is the other type of scleroderma
|
Calcinosis
|
|
Associated with _____ antibody
|
Anti-centromere
|
|
Symptoms of Goodpastures
|
Hemoptysis, hematuria, pulmonary hemorrhages, renal lesions, crescentic glomerulonephritis
|
|
Most common afflicts
|
Men 20-40 y/o
|
|
|
|
|
Endocrine / reproductive
|
|
|
cushing's disease (as compared to Cushing's syndrome) is caused by
|
primary pituitary adenoma secreting ACTH
|
|
how is this different from a primary adrenal hyperplasia / neoplasia causing Cushing's syndrome
|
ACTH↓
|
|
Carcinoid syndrome causes _____ AVCTH secretion
|
ectopic
|
|
The most common cause is
|
iatrogenic (exogenous administration), ACTH↑
|
|
in addition to the moon facies, buffalo hump, what are the cardio and metabolic signs
|
HT
|
|
what is the primary cuase of hyperaldosteronism
|
Conn's syndrome (aldosterone secreting tumor)
|
|
Symptoms
|
HT, hypokalemia, LOW renin level
|
|
TX
|
includes spironolactone
|
|
name some causes of secondary hyperaldosteronism
|
Renal artery stenosis
|
|
mechanism of secondary causes
|
kidney perceives low intravascular volume, results in hyperactive renin-angio system
|
|
thus, renin would be
|
Hign
|
|
____ disease is a primary deficiency of aldosterone and cortisol due to adrenal atrophy
|
Addison's
|
|
patient presents with
|
hypotension and skin hyperpigmentation (↑MSH)
|
|
why hypotension?
|
lack of cortisol leads to hyponatremic volume contraction
|
|
how is Addison's different than secondary deficiency
|
no skin pigmentation in secondary
|
|
____ is the most common tumor of the adrenal medulla in adults
|
Pheo
|
|
what about in kids?
|
neuroblastoma
|
|
note that a neuroblastoma can occur anywhere along
|
the sympathetic chain
|
|
pheos are detected by ___ in urine and ___ in blood
|
VMA, catecholamines
|
|
pheos are assoc. with what other conditions
|
MEN II/III
|
|
TX with
|
phenoxybenzamine (a nonselective, irreversible alpha antagonist)
|
|
what is the rule of 10's that applies to pheos
|
10% malignant, bilateral, extraadrenal, calcify, kids, familial
|
|
MEN Type I is aka
|
Wermer's syndrome
|
|
What are the three-P's
|
Pancreas
|
|
what are the common pancreas afflictions
|
ZE syndrome, insulinomas, VIPomas
|
|
MEN Type II is aka
|
Sipple's syndrome
|
|
what three conditions are prevalent in MEN II
|
medullary carcinoma of thyroid
|
|
how are all MEN syndromes inherited
|
auto. dominant
|
|
how is MEN III different than MEN II
|
oral and intestinal ganglioneuromatosis instead of parathyroid tumor
|
|
pretibial myxedema, diffuse goiter, proptosis and EOM swelling, this patient has
|
graves disease
|
|
what are the labs like in HYPOthyroidism
|
TSH↑, T4↓ and T3↓
|
|
what about in HYPERthyroidism
|
TSH↓, T4↑ and T3↑
|
|
cretinism is a result of
|
lack of iodine
|
|
it is endemic wherever _____ is present
|
endemic goiter
|
|
what do these cretin-kids look like
|
pot-bellies, pale and puffy faced
|
|
what type of coma results from Type I diabetes
|
DKA
|
|
and from Type II?
|
hyperosmolar coma
|
|
insulin deficiency (and glucagon excess leads to what 2 'increases' and what 1 'decrease'
|
Increases: protein catabolism, lipolysis
|
|
↑lipolysis leads to what accumulation
|
↑plasma FFAs, ketonuria, ketonemia
|
|
the ↑protein catabolism leads to
|
↑plasma AA's and nitrogen loss via urine
|
|
the small vessel disease in DM is due to
|
BM thickening
|
|
↑plasma FFas lead to
|
large vessel atherosclerosis, CAD
|
|
cataracts and glaucoma due to
|
sorbitol accumulation
|
|
what lab measures long-term diabetic control
|
HbA1-c
|
|
hyperglycemia increases intracellular sorbitol, thereby ↓ intracellular _____ levels
|
myoinositol
|
|
altered inositol pathway leads to ↓ in cellular ____ activity
|
Na-K-ATPase
|
|
what is the genetic predisposition (strong or weak) of Type I/II DM
|
type I - weak
|
|
which ahs the HLA association
|
Type-I, HLA-DR 3 and 4
|
|
DKA is often triggered by
|
stress (ie infection)
|
|
Kussmaul hyperpnea is
|
rapid, deep breathing
|
|
what type of metabolic acidosis in DKA
|
anion gap
|
|
what are two deadly infections that diabetics get
|
Mucormycosis
|
|
intense thirst and polyuria together with an inability to concentrate urine is known as
|
diabetes insipidus
|
|
central DI is due to
|
lack of ADH
|
|
nephrogenic DI is due to
|
lack of renal response to ADH
|
|
what two drugs can cause DI
|
Lithium
|
|
lab findings: SG & serum osmolarity
|
SG < 1.006 and osmolarity > 290
|
|
TX for central DI
|
intranasal desmopressin (ADH analog)
|
|
TX for nephrogenic DI
|
thiazide, amiloride, indocin
|
|
In contrast to DI, what are the results of SIADH
|
excess water retention, hyponatremia
|
|
note that very low sodium levels can lead to
|
seizures
|
|
which cancer can lead to ectopic ADH secretion
|
small cell lung cancer
|
|
second common cause
|
head trauma
|
|
carcinoid syndrome is caused by carcinoid tumors of neuroendocrine cells, esp. of the ______
|
small bowel
|
|
these tumors secret high levels of ____ that does not get metabolized due to liver metastases
|
serotonin (5HT)
|
|
symptoms?
|
cutaneous flushing and diarrhea, wheezing and carcinoid heart disease
|
|
this is the most common tumor of the _______
|
appendix
|
|
detected by right sided valvular disease and ____ in urine
|
↑5-HIAA
|
|
these carcinoid tumors are TX with
|
octreotide
|
|
what type of tumor is assoc. with ZE syndrome
|
gastrin secreting tumor located in the pancreas
|
|
may be assoc. with
|
MEN-I
|
|
what is the relation to race for osteoporosis
|
whites>blacks>asians
|
|
Type I=
|
post-menopausal
|
|
Type II=
|
senile
|
|
type I caused by
|
↑bone resorption due to ↓estrogen
|
|
pt present with
|
loss of height, kyphosis, vertebral crush
|
|
age onset for type-II
|
>70 y/o
|
|
pt presents with
|
distal radius (Colle's) fractures
|
|
BPH is characterized by nodular enlargement of the ______ lobes of the prostate
|
periurethral (lateral and middle)
|
|
BPH is NOT considered a
|
pre-malignant lesion
|
|
what is a hydatidiform mole
|
a pathologic ovum
|
|
results in swelling of _____ and proliferation of
|
chorionic villi, chorionic epithelium (trophoblast)
|
|
HM is the most common precursor to
|
choriocarcinoma
|
|
marker?
|
high beta-hCG
|
|
how does the uterus appear
|
honeycombed, bunch of grapes
|
|
genotype of a complete mole is
|
46 XX
|
|
and is purely ____ in origin
|
paternal
|
|
there is no ____ actually present
|
fetus
|
|
a partial mole is commonly
|
triploid or tetraploid
|
|
endometriosis results in ____ cysts
|
blood filled, chocolate
|
|
____ is the most common site
|
ovary
|
|
endometrial hyperplasia, on the other hand, is caused by excess
|
estrogen stimulation
|
|
most commonly manifest by
|
vaginal bleeding
|
|
what is the most common gyn. Malignancy
|
endometrial carcinoma
|
|
peak ages
|
55-65
|
|
presents with vag. bleeding and often proceeded by
|
endo. hyperplasia
|
|
risk factors
|
prolonged estrogen use, obesity, diabetes and HTN
|
|
what is the single MOST common tumor of females
|
leiomyoma
|
|
race bias?
|
↑occurrence in blacks
|
|
is it estrogen sensitive?
|
yes, size ↑ in pregnancy and ↓ in menopause
|
|
does it lead to any malignancies?
|
not usually
|
|
however, what is the de novo malig. Counterpart
|
leimyosarcoma
|
|
Appearance
|
bulky with areas of hemorrhage and necrosis
|
|
what hormone is elevated in polycystic ovary disease
|
LH
|
|
where does it come from and what is the effect of the ↑LH
|
peripheral estrogen production and it leads to leads to anovulation
|
|
how does the typical pt present
|
amenorrhea, infertility, obesity, hirsutism
|
|
TX?
|
weight loss, OCP, gonadotropin analogs, surgery
|
|
pt presents with diffuse breast pain and multiple lesions, it is deemed to be fibrocystic disease, is it a risk for carcinoma?
|
not usually, only with the occurrence of atypical cells in epithelial hyperplasia
|
|
____ = a large bulky mass of connective tissue and cysts, breast surface has a leaf-like appearance
|
cystosarcoma phyllodes
|
|
benign or malig.?
|
benign!
|
|
____ = most common <25 y/o, small mobile firm mass with sharp edges, ↑ in size and tenderness w/pregnancy
|
fibroadenoma
|
|
benign tumor of lactiferous ducts that presents with nipple discharge
|
intraductal papilloma
|
|
Now for the malignant tumors:
|
|
|
most common carcinoma?
|
infiltrating ductal
|
|
feels like?
|
firm fibrous mass
|
|
eczematous patches on nipple
|
Paget's
|
|
cheesy consistency of tumor due to central necrosis
|
comedocarcinoma
|
|
common risk factors for breast cancer
|
early menarche (<13 y/o)
|
|
important to know that risk is NOT ↑ by what two common occurrences
|
fibroadenoma
|
|
|
|
|
Cardiovascular
|
|
|
race bias for HTN
|
black>white>asian
|
|
how much is due to essential hypertension
|
90%
|
|
remaining 10% due to
|
mostly renal disease
|
|
pathology of HTN?
|
hyaline thickening and atherosclerosis
|
|
____ is the triad of HTN, proteinuria & edema
|
preeclampsia
|
|
eclampsia is the addition of
|
seizures
|
|
risk ↑ with
|
HTN, diabetes, renal disease, autoimmune disorders
|
|
clinical presentation
|
edema of face, blurry vision, abdominal pain
|
|
TX for pre-eclampsia
|
bed rest, salt restriction, monitor
|
|
TX for eclampsia
|
medical emergency: IV MgSO4 and diazepam
|
|
highest risk sites for atherosclerosis
|
abdominal aorta > coronary artery > politeal artery > carotid artery
|
|
angina occurs when CAD narrowing > ?%
|
>75%
|
|
stabile angina manifests as what kind of pain
|
retrosternal chest pain with exertion
|
|
prinzmetal's is secondary to
|
coronary artery spasm
|
|
MI most often occurs in CAD involving which artery
|
left anterior descending
|
|
sudden cardiac death is within 1 hr and is most often due to
|
arrhythmia
|
|
coronary artery occlusion: LAD > ? > ?
|
LAD > RCA > circumflex
|
|
color of infarcted cardiac tissue 1 day - 7 weeks
|
2-4 days: pallor and hyperemia
|
|
maximum neutrophil immigration around
|
4 days
|
|
macrophages show up around
|
5-10 days (same time as ingrowth of granulation tissue)
|
|
gold-standard of MI detection in first 6 hrs?
|
EKG
|
|
what lab test can be used from 4 hrs up to 7 days
|
troponin-I (most specific)
|
|
____ is the test of choice in the first 24 hrs post-MI
|
CK-MB
|
|
which LDH is also elevated
|
LDH-1
|
|
transmural ischemia on EKG shown as
|
ST elevation
|
|
what about transmural infarct
|
Q-waves
|
|
MOST common complication of MI???
|
arrhythmias (90% of the time)
|
|
4-10 days post-MI, look out for
|
rupture of ventricular free wall, inter-vent. septum, and papillary muscle
|
|
friction rub most common ____ days post-MI
|
3-5 days
|
|
and what is it??
|
several weeks post MI
|
|
what are the ABCDs of systolic dysfunction
|
Alcohol
|
|
the ABCDs above lead to dilated (congestive) cardiomyopathy. xray findings?
|
dilated heart (ballooned)
|
|
Hypertrophy of ____ is often asymmetric involving septum
|
HCM
|
|
is it genetic?
|
yes - 50% cases are familial, inherited AD
|
|
how does LV chamber look on echo?
|
banana shaped
|
|
endomyocardial fibrosis, sarcoidosis and amyloidosis all lead to
|
restrictive / obliterative cardiomyopathy
|
|
name the murmur cause:
|
|
|
crescendo-decrescendo systolic
|
aortic stenosis
|
|
high-pitched 'blowing' diastolic murmur
|
aortic regurgitation
|
|
rumbling-late diastolic murmur, LA>LV pressure during diastole, opening snap
|
mitral stenosis
|
|
high-pitched 'blowing' holosystolic murmur
|
mitral regurgitation
|
|
most frequent valvular lesion (esp. in young women)
|
mitral prolapse
|
|
VSD causes what type of murmur
|
holosystolic murmur
|
|
PDA causes
|
continuous machine-like murmur
|
|
___ are the most common cardiac tumor and 90% occur in the
|
myxomas, atria (mostly LA)
|
|
usually described as a ____ obstruction
|
"ball-valve"
|
|
____ are the most frequent cardiac tumors in kids
|
rhabdomyomas
|
|
in pulmonary congestion due to LV failure, what special type of "cells" do we see
|
heart-failure cells (hemosiderin laden macrophages)
|
|
↓contractility leads to ↓CO leads to ↓arterial volume, what two ways does the body compensate
|
Renin↑ and sympathetic outflow↑
|
|
what compenent in the renin pathway leads to ↑renal vasoconstriction
|
angiotensin II (as does ↑symp. outflow)
|
|
what causes ↓GFR
|
renal vasoconstriction
|
|
this causes?
|
↓urine excretion of NA and water
|
|
what is predisposed by Virchow's triad
|
DVTs
|
|
name the triad
|
stasis, hypercoagulability, endothelial damage
|
|
pt has a new murmur, anemia and fever....what do you think
|
bacterial endocarditis
|
|
three classic lesions involved
|
Roth spots (round whit spots on retina surrounded by hemorrhage)
|
|
what other sign is on nailbed
|
splinter hemorrhages
|
|
2. subacute
|
|
|
subacute involves
|
smaller lesions on preciously abnormal valves
|
|
which is assoc. w/dental procedures
|
subacute
|
|
what is the histologic feature of RHD
|
Aschoff bodies
|
|
other key indicators?
|
migratory polyarthritis, erythema marginatum
|
|
any elevated titers?
|
ASO titers
|
|
how does tertiary syphilis cause heart disease
|
disrupts vasa-vasorum of aorta with consequent dilatation of aorta and valve ring
|
|
any particular region of aorta affected
|
aortic root / ascending aorta
|
|
how does the aorta appear?
|
tree-bark appearance
|
|
Burger's Disease aka
|
smoker's disease
|
|
signs/symptoms
|
intermittent claudication, superficial nodular phlebitis, Raynaud's, severe pain
|
|
TX?
|
quit smoking
|
|
____ aka Pulseless Disease
|
Takayasu's arteritis
|
|
why 'pulseless'?
|
thickening of aortic arch and great vessels leads to weak upper extremity pulses
|
|
primarily affects
|
young, Asian females
|
|
FAN MY SKIN ?
|
Fever, Arthritis, Night sweats, MYalgia, SKIN nodules
|
|
what are the findings in Temporal Arteritis
|
Elderly, ↑ESR, unilateral headache, jaw claudication, impaired vision
|
|
TX?
|
responds well to steroids
|
|
P-ANCA
|
polyarteritis nodosa
|
|
_______ is characterized by focal necrotizing vasculitis, necrotizing granulomas in the lung and upper airway and necrotizing glomerulonephritis
|
Wegener's granulomatosis
|
|
TX
|
cyclophosphamide, corticosteroids, MTX
|
|
ethanol is converted to acetaldehyde, how does this lead to fatty change?
|
acetaldehyde to acetate to acetyl-CoA to increased FA synthesis to fatty liver
|
|
what ratio is ↑
|
NADH/NAD
|
|
name two neurological manifestations of alcoholism
|
flapping tremor of hands
|
|
what causes Wernicke-Korsakoff syndrome
|
Thiamine (B1) deficiency
|
|
classic triad of symptoms (of Wernicke's encephalopathy)
|
psychosis, opthalmoplegia, ataxia
|
|
Progression to Korsakoff syndrome involves
|
memory loss, confusion and confabulation
|
|
argyl-robertson pupil pathognomonic for
|
tertiary syphilis
|
|
what do you see?
|
constricts with accomodation but not reactive to light
|
|
ARP
|
accomodation reaction present
|
|
primary amyloidosis seen with what 2 conditions
|
multiple myeloma, waldenstroms macroglobulinemia
|
|
histology?
|
apple-green birefringence with Congo Red stain
|
|
link to Alzheimer's?
|
beta-amyloid deposition in cortex
|
|
whereas, islet cell amyloid deposits seen in
|
DM Type II
|
|
granuloma with giant cells aka
|
Aschoff Bodies
|
|
Aschoff bodies AND _____ are found in RHD
|
Anitschkow's cells
|
|
Think of two ____ with RHeumatic heart disease
|
RHussians (ascoff and anitschkow)
|
|
Auer rods pathognomic for
|
acute promyelocytic leukemia
|
|
what are they?
|
cytoplasmic inclusions in granulocytes and myeloblasts
|
|
presence of Casts in urine indicates that hematuria / pyuria is or _______ origin
|
renal
|
|
RBC casts =
|
bladder cancer
|
|
WBC casts =
|
acute cystitis
|
|
____ casts often seen in normal urine
|
hyaline
|
|
____ casts are often seen in chronic renal failure
|
waxy
|
|
ESR best used to DX and monitor
|
temporal arteritis and polymyalgia rheumatica
|
|
____ = TB granulomas with lobar or perihylar LN involvement
|
Ghon complex
|
|
indicative of
|
primary infection or exposure
|
|
arcus senilis and xanthomas indicative of
|
hyperlipidemia
|
|
Psammoma bodies seen in (PSaMM)
|
Papillary adenocarcinoma of thyroid
|
|
helmet cell or schistocyte indicates
|
DIC, traumatic hemolysis
|
|
Teardrop shaped RBC indicates
|
myeloid metaplasia with myelofibrosis
|
|
Target cells seen in
|
thalassemias, liver disease
|
|
Poikilocytes =
|
nonuniform shapes in TTP/HUS, DIC
|
|
Burr cells =
|
TTP/HUS
|
|
PAIR pneumonic for HLA-B27
|
Psoriasis
|
|
____ = distinctive tumor giant cell seen in Hodgkin's
|
Reed-Sternberg
|
|
Describe
|
large, bi-nucleate or bi-lobed with the two halves being mirror images (Owl-eyed)
|
|
sufficient or necessary for DX
|
necessary but NOT sufficient
|
|
of the 4 types of Hodgkin's, which is more prevalent in women
|
nodular sclerosis
|
|
what is it's PX
|
excellent
|
|
____ = presumptive evidence of malignant visceral neoplasm (even medical students can detect this!)
|
Virchow's Node
|
|
|
|
|
Renal
|
|
|
which glumerular pathology is characterized by hematuria, HT, oliguria and azotemia
|
nephritic syndrome (i=inflammation)
|
|
what do glomeruli look like under LM in post-strep glomerulonephritis
|
enlarged and hypercellular, PMNs, lumpy-bumpy
|
|
most frequently seen in kids, what are the presenting symptoms
|
peripheral and peri-orbital edema
|
|
TX?
|
resolves spontaneously
|
|
what type of glomerulonephritis has crescent-moon shape under LM
|
rapidly progressing (crescentic)
|
|
what does the pt have if they present with hematuria and hemoptysis
|
goodpastures
|
|
IF pattern?
|
linear
|
|
any antibodies?
|
anti-GBM
|
|
which glomerulonephritis has Tram-Track appearance on EM
|
membranoproliferative
|
|
which has mesangial deposits of IgA
|
Berger's IgA nephropathy
|
|
|
|
|
what glomerular pathology presents with massive proteinuria, hypoalbuminemia, edema, hyperlipidemia (due to loss of proteins)
|
nephrotic (o=proteinemia)
|
|
what's the common cause of adult nephrotic syndrome
|
membraneous glomerulonephritis
|
|
what's seen on LM and EM
|
LM: diffuse capillary and BM thickening
|
|
what's the most common cause of childhood nephrotic syndrome
|
minimal change disease (good PX)
|
|
responds well to
|
steroids
|
|
what's seen on EM?
|
effacement of foot processes
|
|
Kimmelstiel-Wilson lesions seen in
|
diabetic nephropathy
|
|
wire-loop patterns?
|
SLE
|
|
name the 4 major types of kidney stones
|
calcium
|
|
which comprises the majority of stones
|
calcium
|
|
xray findings?
|
stones are radiopaque
|
|
recurrence rate?
|
high
|
|
____ are the second most common and are formed in alkaline urine by urease (+) bugs
|
ammonium
|
|
name some bugs
|
proteus vulgaris
|
|
which go hand-in-hand with gout
|
uric acid stones
|
|
often seen in conjunction with what type of disease
|
those with ↑ cell proliferation and turnover, i.e. leukemia and myeloproliferative disorders
|
|
xray findings?
|
radiolucent (so are cystine stones)
|
|
most common renal malignancy?
|
renal cell carcinoma
|
|
most common in
|
men, 50-70 y/o
|
|
assoc. with which syndrome? which chromosome?
|
von Hippel Lindau, C3
|
|
presenting symptoms
|
hematuria, flank mass, pain, secondary polycythemia
|
|
spread?
|
via IVC to blood!
|
|
most common renal malignancy of kids
|
Wilm's tumor
|
|
gene involved?
|
deletion of tumor suppressor gene WT-1 on C11
|
|
what is the WARG complex
|
Wilm's tumor
|
|
tumor in urinary tract is probably
|
transitional cell carcinoma
|
|
presents with
|
painless hematuria
|
|
exposure risks?
|
phenacetin, analine dyes, cyclophosphamide, smoking
|
|
how do you calculate anion gap
|
Gap = NA - (Cl+HCO3)
|
|
normal range
|
8-12
|
|
if elevated, what's the pneumonic
|
MUD PILES
|
|
acute renal failure is most often due to
|
hypoxia
|
|
and chronic?
|
HTN and diabetes
|
|
Renal Failure: how do the following result;
|
|
|
Anemia
|
failure to make EPO
|
|
renal osteodystrophy
|
failure of active Vit-D production
|
|
Hyperkalemia
|
↓urine output
|
|
metabolic acidosis
|
↓acid secretion
|
|
Uremia
|
↑BUN, creatinine
|
|
CHF, pulmonary edema
|
↓Na and water loss
|
|
|
|
|
Tetany caused by ↓?
|
Calcium
|
|
what else is caused by ↓Ca
|
rickets, osteomalacia
|
|
what else is deficient in rickets / osteomalacia
|
phosphate
|
|
|
|
|
metabolic acidosis: what parameter is massively↓
|
HCO3-
|
|
how are pH and pCO2
|
both ↓
|
|
causes?
|
DKA, diarrhea, lactic acidosis, aspirin OD, acetazolamide OD
|
|
compensatory response
|
hyperventilation to ↓acidity
|
|
parameters for respiratory acidosis
|
pH↓ pCO2↑↑↑ HCO3-↑
|
|
Causes
|
COPD, airway obstruction
|
|
Compensation
|
renal HCO3- reabsorption
|
|
parameters for respiratory alkalosis
|
pH↑ pCO2↓↓ HCO3-↓
|
|
Causes
|
hyperventilation, high altitude
|
|
Compensation
|
renal HCO3- secretion
|
|
parameters for metabolic alkalosis
|
pH ↑ pCO2↑ HCO3-↑↑
|
|
Causes
|
vomiting
|
|
Compensation
|
hypoventilation
|
|
henderson-hasselbach equation
|
pH=pKa +log [HCO3-] / [0.03 pCO2]
|
|
what's the blood pH cutoff for acidosis / alkalosis
|
pH>7.4 = alkalosis, pH<7.4 for acidosis
|
|
what's the pCO2 value to know
|
40
|
|
pt presents with pH of 7.3 and pCO2 of 51, what is there classification
|
resp. acidosis
|
|
pt with pH=8.0, pCO2 of 15
|
resp. alkalosis
|
|
pt with pH=7.28 and pCO2=30
|
metabolic acidosis w/compensation
|
|
give two values that lands you in metabolic alkalosis
|
pH>7.4
|
|
what's the next step after discovering that a pt is in metabolic acidosis
|
determine if it is anion gap acidosis
|
|
if not anion gap caused, what's a common cause
|
diarrhea, glue sniffing renal tubular acidosis
|
|
30y/o chemical worker presents with MPTP exposure – what neurotransmitter is depleted?
|
Dopamine
|
|
A woman taking tetracycline shows photosensitivity – what are the clinical manifestations?
|
Rash on sun exposed regions of the body?
|
|
Young girl with congenital valve disease is given PCN prophylactically but develops endocarditis – what should you give her now?
|
Beta-lactamase resistant PCN.
|
|
Non-diabetic pt presents with hypoglycemia but low levels of C peptide – what is the diagnosis?
|
Surreptitious insulin injection
|
|
Black man who goes to Africa develops anemia after taking prophylactic medicine – what is the likely enzyme deficiency?
|
G6PD
|
|
27 y/o female with a hx of psychiatric illness develops urinary retention while on a neuroleptic – what medication would help treat this?
|
bethanechol
|
|
A farmer presents with dyspnea, salivation, miosis, diarrhea, cramping, and blurry vision – what is the likely cause and mechanism of action?
|
Insecticide poisoning, inhibition of actylcholinesterase
|
|
55yo man undergoing tx for BPH has decreased levels of testerone and DHT, gynecomastia, and edema – what is the drug?
|
Estrogen(DES)
|
|
Farmer with a recent kidney transplant is on cyclosporine for immunosuppression and now requires oral meds for candidiasis? What medication given concurrently would result in cyclosporine toxicity?
|
Ketoconazole
|