Usmle Pathology

Front 1

Antithrombogenic substances

Back 1

Prostacyclin (PGI2), nitric oxide, tissue plasminogen activator, thrombomodulin

Front 2

Steps in hemostasis

Back 2

1. Endothelial injury relases tissue factor (activates factor VII, extrinsic pathway); exposure of thrombogenic subendothelial collagen activates factor XII (intrinsic pathway), release of vWF; decreased synthesis of antithrombogenic substances; 2. Platelet adhesion to vWF through glycoprotein Ib; 3. Platelet activation with degranulation and synhesis of TXA2 and ADP (aggregators); 4. Aggregation mediated by TXA2 and ADP and fibrinogen/Gp IIb-IIIa

Front 3

Bernard Soulier syndrome

Back 3

Autosomal recessive. Deficiency of platelet GPIb. Defective platelet adhesion

Front 4

Glanzman thrombastenia

Back 4

Deficiency of Gp IIB-IIIa; defective platelet aggregation

Front 5

Immune thrombocytopenic pupura

Back 5

Antiplatelet antibodies and destruction in spleen by macrophages (bing IgG coated platelets via Fc receptor). Thrombocytopenia, prolonged bleeding time, normal PT and PTT. Petechiae, ecchymoses, menorrhagia, nosebleeds. Present in Wiskot-Aldrich

Front 6

Thrombotic thrombocytopenic purpura

Back 6

Platelet thrombi with scant fibrin with no activation of coagulation system. Fever, thrombocytopenia, hemolytic anemia, neurologic symptoms, renal failure. Increased bleeding time, normal PT/PTT, schistocytes

Front 7

Hemolytic uremic syndrome

Back 7

Gastroenteritis with bloody diarrhea, fever, thrombocytopenia, renal failure, hemolytic anemia. Produced by verotixin-producing E. coli 0157

Front 8

Activation of coagulation system

Back 8

Intrinsic pathway (Factor XII): exposure to subendothelial collagen; Extrinsic pathway (factor VII): tissue thromboplastin by endothelium

Front 9

Kinin cascade

Back 9

Hageman factor converts prekallikrein into kallikrein. HMWK is converted to bradykinin by kallikrein

Front 10

Fibrinolytic system cascade

Back 10

Kallikrein activates plasminogen into plasmin which inhibits fibrin from coagulation cascade

Front 11

Vitamin K-dependant factors

Back 11

Factors II, VII, IX, X. Both intrinsic and extrinsic paths need vitamin k-dependant gamma carboxylation

Front 12

Prothrombin time (PT)

Back 12

Tests extrinsic and common paths. Factors V, VII, X, prothrombin and fbrinogen. Used to monitor warfarin therapy

Front 13

MOA of warfarin

Back 13

Blocks epoxide reductase (activates vitamin K). Takes 3-4 days for effect due to long half life of previously carboxylated factors which are still circulating

Front 14

Partial thromboplastin time (PTT)

Back 14

Tests intrinsic and common paths. Factors XII, XI, IX, VIII, X, V, prothrombin, fibrinogen. Used to monitor heparin

Front 15

DIC

Back 15

Thrombocytopenia, prolonged PT/PTT, decreased fibrinogen, elevated D-dimers

Front 16

Hemophilia

Back 16

Deficiency of factor VIII or IX. X-linked recessive (affects males). Bleeding at circumcision, hemarthrosis, easy bruising and hematomas. No petechiae or ecchymoses. Normal platelets and bleeding time, normal PT, prolonged PTT (intrinsic path coagulopathy)

Front 17

Increased PT and PTT

Back 17

Vitamin K deficiency, liver disease

Front 18

Von Willenbrand disease

Back 18

bleeding from mucous membranes, prolonged bleeding time, normal PT/PTT, abnormal response to ristocetin

Front 19

Auto mechanics

Back 19

Carbon monoxide poisoning. Decreased SaO2, cherry red color of skin, headache. Rx.: 100% O2

Front 20

Firefighters

Back 20

Carbon monoxide poisoning. Decreased SaO2, cherry red color of skin, headache. Rx.: 100% O2

Front 21

Wood stoves and space heaters

Back 21

Carbon monoxide poisoning. Decreased SaO2, cherry red color of skin, headache. Rx.: 100% O2

Front 22

Pesticide industry

Back 22

Organophosphate poisoning (acetylcholinesterase inhibitors) and arsenic. Lacrimation, salivation, miosis, weakness. Rx.: atropine

Front 23

Meat packing industry

Back 23

Plyvinyl chloride (PVC). Hepatic angiosarcoma

Front 24

Coal workers

Back 24

Anthracosis. Pulmonary fibrosis and respiratory distress

Front 25

Insulation workers

Back 25

Asbestosis. Interstitial lung fibrosis, fibrous pleural plaques, brnchogenic CA, mesotheliomas, recurrent pleural effusions, dyspnea, pleuritic chest pain

Front 26

Demolition workers

Back 26

Asbestosis. Interstitial lung fibrosis, fibrous pleural plaques, brnchogenic CA, mesotheliomas, recurrent pleural effusions, dyspnea, pleuritic chest pain

Front 27

Construction workers

Back 27

Asbestosis. Interstitial lung fibrosis, fibrous pleural plaques, brnchogenic CA, mesotheliomas, recurrent pleural effusions, dyspnea, pleuritic chest pain

Front 28

Shipyard workers

Back 28

Asbestosis. Interstitial lung fibrosis, fibrous pleural plaques, bronchogenic CA, mesotheliomas, recurrent pleural effusions, dyspnea, pleuritic chest pain

Front 29

Dry cleaners

Back 29

Carbon tetrachloride (CCL4). Liver centrilobular necrosis

Front 30

Rubber/chemical workers

Back 30

Benzene. Aplastic anemia, leukemia

Front 31

Battery factory workers

Back 31

Lead poisoning. MOA: denatures ferrochelatase, (microcytic anemia with ringed sideroblasts), denatures ALA dehydrase. Clinical features: depositis in epiphysis, lethargy, cognitive impairment, cerebral edema, lead colic, basophilic stipling. Dx.: blood lead levels

Front 32

Plumbers

Back 32

Lead poisoning. MOA: denatures ferrochelatase, (microcytic anemia with ringed sideroblasts), denatures ALA dehydrase. Clinical features: depositis in epiphysis, lethargy, cognitive impairment, cerebral edema, lead colic, basophilic stipling. Dx.: blood le

Front 33

Pottery paint

Back 33

Lead poisoning. MOA: denatures ferrochelatase, (microcytic anemia with ringed sideroblasts), denatures ALA dehydrase. Clinical features: depositis in epiphysis, lethargy, cognitive impairment, cerebral edema, lead colic, basophilic stipling. Dx.: blood le

Front 34

Combustion of polyurethane foam during fires

Back 34

Cyanide poisoning. Blocks cytochrome oxidase. Hypoxia. Rx.: nitrites (form methhemoglobin which binds cyanide before it reaches tissues), thiosulfate bind cyanide forming thiocyanate

Front 35

Dental amalgams

Back 35

Mercury poisoning. Intention tremors, dementia, delirium

Front 36

Insecticides

Back 36

Mercury poisoning. Intention tremors, dementia, delirium

Front 37

Hat-making industry

Back 37

Mercury poisoning. Intention tremors, dementia, delirium

Front 38

Cancers produced by cigarette smoke

Back 38

Lung, oral cavitiy, pharynx, larynx, esophagus, pancreas, kidney

Front 39

Cardiovascular disease due to cigarette smoke

Back 39

Atherosclerosis (major risk factor), CAD, AMI, Buerger disease

Front 40

Respiratory disease due to cigarette smoke

Back 40

Chronic bronchitis, emphysema, asthma

Front 41

Diseases associated with alcohol

Back 41

Thiamine deficiency (Wernicke-Korsakoff), macrocytic anemia (folate deficiency), Mallory-Wiess, Boerhaave's syndrome, cirrhosis, esophageal varices, acute pancreatitis, congestive cardiomyopathy, hyperlipidemia

Front 42

Down syndrome

Back 42

Trisomy 21 due to meiotic nondisjunction (95%) or robertsonian translocation (4%); Severe mental retardation, mongoloid features, brushfeld spots, simian crease, heart defects, duodenal atresia, Hirchsprung, ALL, Alzheimer's by age 40

Front 43

Edward syndrome

Back 43

Trisomy 18. Mental retardation, low set ears, micrognathia, heart defects, overlapping flexed fingers, rocker-bottom feet

Front 44

Patau syndrome

Back 44

Trisomy 13. Mental retardation, cleft lip or palate, cardiac defects, renal defects, microcephaly, polydactyly

Front 45

Cri du chat

Back 45

5p deletion. Cat-like cry, mental retardation, heart defects, microcephaly

Front 46

Klinefelter syndrome

Back 46

47XXY. Male hypogonadism, testicular atrophy, infertility, female distribution of hair, gynecomastia, elevated FSH/LH ratio, low levels of testosterone

Front 47

Turner syndrome

Back 47

45X0. Female hypogonadism, no barr body, no secondary sex characteristics, short stature, widely spaced niples, gonadal dysgenesis, amenorrhea, infertility, hypothyroidism, preductal coarctation of the aorta, bicuspid aortic valve

Front 48

Female hermaphrodite

Back 48

46XX. Female internal organs, virilized external genitalia. Cause: congenital adrenal hyperplasia, androgen-producing tumor

Front 49

Male hermaphrodite

Back 49

46XY. Testes present, female genitalia, testicular feminization. Cause: androgen insensitivity syndrome

Front 50

CCystic Phibrosis

Back 50

Chloride Channel protein defect, DP508 on chromosome 7. Recurrent pseudomona/staph infections, chronic bronchitis, bronchiectasis, atrophy of pancreatic ducts, pancreatic insuficiency, fat malabsorbtion, steatorrhea, infertility, meconium ileum, elevated NaCl sweat test

Front 51

PKU

Back 51

Deficiency of phenylalanine hydroxylase. Mental retardation by 6 months, light-colored hair and skin, musty odor. Avoid aspartame, monitor pregnancy

Front 52

Alkaptonuria

Back 52

"Black Homo" homogentisic acid oxidase deficiency. Black urine, black cartilage

Front 53

Albinism

Back 53

Tyrosinase deficiency. Increased risk of squamous carcinoma, no melanin

Front 54

Von Gierke disease

Back 54

Glucose 6 phosphatase deficiency. Hepatomegaly, fasting hypoglycemia, hyperuricemia

Front 55

Pompe disease

Back 55

Lysosomal alpha 1-4 glucosidase deficiency. Hepatomegaly, muscle hypotonia, cardiomegaly

Front 56

McArdle disease

Back 56

Myophosphorylase deficiency. Excersice-induced muscle cramps

Front 57

Tay SaX

Back 57

Hexosaminidase A deficiency with acumulation of glangliocerebroside GM2. Mutation of HEXA gene on chromoseme 15. Cherry-red spot on retina

Front 58

Nieman PickS

Back 58

Sphingomyelinase deficiency. Cherry-red spot on retina, hepatosplenomegaly, zebra bodie on EM

Front 59

Gaucher disease

Back 59

Glucocerebrosidase deficiency. In adulthood, hepatosplnomegaly, hypersplenism, lymphadenopathy, osteoporosis

Front 60

Mucopolysaccharidosis

Back 60

Glycosaminoglycans acumulation. Mental retardation, cloudy cornea, coarse facial features, hepatosplenomegaly, skeletal deformities

Front 61

Familial hypercholesterolemia

Back 61

Mutation on LDL receptor gene on chromosome 19. Xanthomas, xanthelasmas, atherosclerosis

Front 62

Marfan syndrome

Back 62

Mutation of fibrillin gene on 15q. Tall, thin with big extremities, hyperextensible joints, pectus excavatum, ectopia lentis, disecting aortic aneurysm, aortic insuficiency, mitral prolapse

Front 63

Ehlers-Danlos

Back 63

Hyperxtensible skin and joints. Collagen gene defects

Front 64

Menkes disease

Back 64

Mutation in Cu+ efflux protein gene. High concentration of Cu+ that cant be released. Associated with Ehlers-Danlos type 9

Front 65

Neurofibromatosis type 1

Back 65

Von Recklinghousen disease (has 17 letters). NF-1 tumor suppressor gene mutation on chromosome 17. Normal gene product neurofibromim inhibits p21 ras oncoprotein. Neurofibromas, café-au-lait spots, Lisch nodules (pigmented iris hamartomas)

Front 66

Neurofibromatosis type 2

Back 66

NF-2 tumor suppressor gene mutation on chromosome 22. Bilateral acoustic neuromas, café-au-lait spots, pheochromocytoma

Front 67

Von Hippel Lindau disease

Back 67

Mutation on VPL tumor suppressor gene on chromosome 3p. Retinal hemangioblastoma, hemangioblastoma of cerebellum, brainstem and spinal cord, cysts of the liver, pancreas and kidneys, bilateral renal carcinomas

Front 68

Fragile X syndrome

Back 68

X-linked dominant mutations on FMR-1 gene. CGG triplet repeats. Retardation elongated face with large jaws, large everted ears, macroorchidism

Front 69

Huntington disease

Back 69

CAG triplet repeats of huntington gene produces neurotoxic protein. Progressive dementia, choreiform movements

Front 70

Prader-Willi syndrome

Back 70

Deletion of paternal 15q. Mental retardation, obesity, hypogonadism, hypotonia

Front 71

Angelman syndrome

Back 71

deletion of maternal 15q. Retardation, seizures, ataxia, inapropriate happy-puppet laughter

Front 72

Homocystinuria

Back 72

Cysthathione synthase deficiency. Resembles Marfan. Ectopic lens, arachnodactyly, eunuchoid proportions osteoporosis, atherosclerosis, DVT

Front 73

Classic 21B-Ohase deficiency

Back 73

Hypovolemia, hyponatremia, female pseudohermaphrodite, hirsutism (increased 17 KS), skin hyperpigmentation (high ACTH increases melanin). Labs: increased serum 17OH-progesterone, hyperkalemia, metabolic acidosis, increased 17KS, decreased 17OHCS, hypocortisolism

Front 74

Non-classic 21B-Ohase deficiency

Back 74

Increase in 17 KS, hirsutism, no salt loss, acne in females, secondary amenorrhea. Labs: increased 17OH pregenterone

Front 75

Classic 11OHase deficiency

Back 75

Salt retention/hypertension (11-deoxycorticosterone), increased 17KS (virilization), skin hyperpigmentation

Front 76

17OHase deficiency

Back 76

Salt retention, hypertension, (aldosterone), female hypogonadism (decreased 17KS), male pseudohermaphrodite (low 17KS). Labs: low 17KS, 17OHCS, hypocortisolism, increased ACTH

Front 77

Clinical features of SLE

Back 77

Type II and III hypersensitivity reactions. Pancytopenia, arthritis, butterfly rash, diffuse proliferative glomerulonephritis, Libman-Sacks endocarditis. ANA, anti-DNA, anti-Sm, anti-histone (drug-induced lupus). Rx.: steroids

Front 78

Sjogren syndrome

Back 78

Autoantibodies against lacrimal and salivary glands. Keratoconjunctivitis, corneal ulcers (dry eyes), xerostomia (dry mouth). Anti-Ro (SS-A), anti-La (SS-B). 15% of rheumatoid arthritis patients have Sjogren.

Front 79

Mikulicz syndrome

Back 79

Enlargement of the salivary and lacrimal glands associated with Sjogren syndrome

Front 80

Diffuse scleroderma

Back 80

Fibroblast stimulation and deposition of collagen in the skin and internal organs. Anti-DNA topoisomarease I (Scl-70) (helicase). Skin involvement, dysphagia, malabsorption, pulmonary fibrosis (dyspnea), cardiac fibrosis (arrhythmias), kidney fibrosis (renal failure)

Front 81

Localized scleroderma (CREST)

Back 81

Fibroblast stimulation and deposition of collagen. Anti-centromere antibodies. Calcinosis, Raynaud, esophageal dysmoility, sclerodactyly, telangiectasia.

Front 82

Bruton's agammaglobulinemia

Back 82

X-linked recessive. No B cells, No Igs. Recurrent staph, haemophilus and strep infections after 6 months. Increased pre-B cells. Mutation of B-cell Bruton tyrosine kinase (btK).

Front 83

Common variable immunodeficiency

Back 83

B-cell maturation defect. Hypogammaglubulinemia, recurrent bacterial infections, giardia lamblia.

Front 84

DiGeorge syndrome

Back 84

Failure to develop 3rd and 4th pharyngeal puches results in absence of parathyroid and thymus glands, hypocalcemia, tetany, T-cell deficiency, recurrent viral infections, heart defects, chronic candidiasis

Front 85

SCID

Back 85

B and T cell deficiency due to mutation of IL-2 receptor (x-linked), adenosine deaminase deficiency (AR) or failure to make MHC II. Recurrent infections and susceptibility to candida, CMV and p. carinii

Front 86

Wiskot-Aldrich syndrome

Back 86

X-linked recessive. "WIPE": recurrent infections, thrombocytopenic purpura, eczema, risk of lymphomas, low IgM

Front 87

Ataxia-Telangiectasia

Back 87

Ataxia, spider angiomas, low IgA, defect of DNA repair enzyme

Front 88

Chronic granulomatous disease

Back 88

Low NADPH oxidase. Recurrent catalase+ infections, negative nitroblue tetrazolium test.

Front 89

Leukocyte adhesion deficiency

Back 89

Defect of CD-18 (LFA-1 beta chain), no pus formation, failure of umbilical cord to detach

Front 90

Chediak-Higashi

Back 90

Defect in microtubules with no phagocytosis by lysosome. Partial albinism, peripheral neuropahty, recurrent infections

Front 91

Hereditary angioedema

Back 91

Edema at mucosal surfaces. Defect of C1-INH (esterase inhibitor). Decreased C1, C2, C4

Front 92

Hyper IgM

Back 92

Defect of CD-40L on T-lymphocytes. No isotype switching, increased IgM

Front 93

MHC-I deficiency

Back 93

Normal CD4, no CD8. Failure of TAP-1 to transport peptides to MHC-I groove

Front 94

Amyloid stains

Back 94

Stains red with Congo-red stain then apple green birefringence under polarized light

Front 95

Signs and symptoms of amyloidosis

Back 95

Nephrotic syndrome, renal failure, arrythmias, CHF, hepatosplenomegaly, macroglosia

Front 96

Mediators of leukocyte margination

Back 96

Selectins mediate margination. P and E selectins on endothelium bind Sialyl-Lewis on leukocyte; GlyCAM/CD34 on endothelium binds L-selectin on leukocyte

Front 97

Mediators of leukocyte adhesion

Back 97

Integrins mediate adhesion. ICAM, VCAM on endothelium bind LFA-1 and VLA on leukocyte

Front 98

Regulators of leukocyte margination and adhesion

Back 98

Histamine upregulates P-selectin. IL-1 and TNF induce E-selectin, ICAM and VCAM. Chemotactic agents cause conformational change of LFA-1

Front 99

Leukocyte adhesion deficiency

Back 99

Defect of CD18 (beta chain subunit of LFA-1 integrin on leukocytes). Recurrent infections, no pus formation, failure of umbilical cord to detach

Front 100

Chemotactic factors

Back 100

N-formyl methionine, leukotriene B4, C5a, IL-8

Front 101

Opsonins

Back 101

Fc portion of IgG, c3b, C reactive protein

Front 102

Chediak-Higashi syndrome

Back 102

Defect of microtubule polymerization causes defect in chemotaxix and degranulation. Partial albinism, peropheral neuropathy

Front 103

CGD

Back 103

NADPH oxidase deficiency. No production of superoxide for respiratory burst. Recurrent catalase+ infections, negative nitroblue tetrazolium test

Front 104

Arachidonic acid products

Back 104

AA produced by phospholipase A2 (inhibited by steroids). Produces leukotrienes, prostaglandins and thromboxane A2

Front 105

Lypoxigenase pathway

Back 105

Arachidonic acid is converted to leukotrienes by 5-lypoxigenase. LTB4 --> chemotaxis. LTC4, D4, E4 --> bronchoconstriction

Front 106

Cycloxigenase pathway

Back 106

Arachidonic acid is converted to TXA2 and prostaglandins (NSAIDs block). TXA2 --> vasoconstriction, platelet aggregator. PGI2, PGE2, PGF2

Front 107

Mediators of vasodilation

Back 107

Histamine , bradikinin, PGI2, PGD2, E2, F2

Front 108

Mediators of pain

Back 108

Bradikinin, PGE2

Front 109

Mediators of increased permeability

Back 109

Histamine, Bradikinin

Front 110

Mediators of vasoconstriction

Back 110

TXA2, LTC4, D4, E4

Front 111

Mediators of bronchoconstriction

Back 111

LTC4, D4, E4, bradikinin

Front 112

Mediators of fever

Back 112

IL-1, PGD2, E2, F2

Front 113

Anaphilotoxins

Back 113

C3a, C5a. Directly stimulate histamine release from basophils, mast cells and platelets

Front 114

C3b

Back 114

Opsonin; neutrophils, macrophages and monocytes have C3b receptros

Front 115

Bradikinin synthesis and actions

Back 115

Synthesized from activation of prekalikrein by factor XII (Hageman). Kalikrein cleaves HMWK into bradikinin. Vasodilator, increased permeability, brnchoconstrictor, pain

Front 116

PGE2

Back 116

Vasodilation in kidneys, increases renal blood flow, increases gastric mucosal blood flow (mucoprotection), activates osteoclasts, fever, pain, maintains ductus arteriosus

Front 117

Prostacyclin (PGI2)

Back 117

Vasodilation and inhibits platelet aggregation

Front 118

IL-1

Back 118

Stimulates PGE2 synthesis in hypothalamus --> fever; B-cell stimulation to synthesize Ig; osteoclast activation (released by osteoblasts under PTH stimulation); lytic bone lessions of multiple myeloma; increases adhesion molecules in endothelium; increases acute phase reactants

Front 119

Hageman factor

Back 119

Activates intrinsic coagulation system, kinin cascade and fibrinolytic system

Front 120

PGF

Back 120

Uterine muscle contraction (cause pf primary amenorrhea)

Front 121

gamma interferon

Back 121

Produced by CD4 TH1 cells and NK cells. Activates macrophages; antiviral properties; class I and class II antigens; increases IL-2, IL-12 production by CD4 cells

Front 122

IL-2

Back 122

Produced by CD4 cells. T cell growth factor. Promotes B cell and NK cell proliferation

Front 123

IL-6

Back 123

Synthesis of acute phase reactants

Front 124

Factors that increase adhesion molecule synthesis

Back 124

C5a, LTB4, IL-1, TNF

Front 125

Key cells in acute and chronic inflamation

Back 125

Acute: neutrophil has IgG and C3b receptros; Chronic: macrophage has receptors for IgG and C3b, process antigen and secrete IL-1, IL-12 and TNF

Front 126

Chronic granulomatous inflamation

Back 126

Epitheloid cells and multinucleated giant cells surrounded by a rim of lymphocytes with central caseous necrosis

Front 127

Type I collagen

Back 127

skin, bones, tendons and most organs

Front 128

Type II collagen

Back 128

Cartilage and vitreous humor

Front 129

Type III collagen

Back 129

granulation tissue

Front 130

Type IV collagen

Back 130

basement membranes

Front 131

Composition of basement membranes

Back 131

Has negative charge. Collagen type IV, proteoglycans (heparan sulfate), laminin, fibronectin

Front 132

Nitrosamines

Back 132

Gastric CA, Esophagus

Front 133

Cigarette smoke

Back 133

Larynx, ling, renal cell carcinoma, transitional cell carcinoma

Front 134

Polycyclic aromatic hydrocarbons

Back 134

Bronchogenic CA

Front 135

Asbestos

Back 135

Bronchogenic CA, mesothelioma

Front 136

Chromium and nickel

Back 136

Bronchogenic CA

Front 137

Arsenic

Back 137

Squamous cell CA of skin and lung, angiosarcoma of liver

Front 138

Vinyl chloride (PVC)

Back 138

Liver angiosarcoma

Front 139

Alkylating agents

Back 139

Leukemia, lymphoma

Front 140

Benzene

Back 140

Leukemia, lymphoma

Front 141

Napthalene

Back 141

Bladder CA

Front 142

CCL4

Back 142

Liver centrilobular necrosis

Front 143

HTLV-1

Back 143

Adult T-cell leukemia

Front 144

HBV, HCV

Back 144

Hepatocellular CA

Front 145

EBV

Back 145

Burkitt's lymphoma, nasopharyngeal CA

Front 146

HPV

Back 146

Cervical CA (16, 18)

Front 147

HHV-8

Back 147

Kaposi sarcoma

Front 148

hst-1 & int-2

Back 148

Cancer of stomach, breast, bladder and melanoma; produces growth factors; overexpression

Front 149

sis

Back 149

Astrocytoma; produces PDGF; overexpression

Front 150

erb-B1

Back 150

SCC of lung; produces EGF receptor; overexpression

Front 151

erb-B2

Back 151

Breast, ovary and lung CA; produces EGF receptor; amplification

Front 152

erb-B3

Back 152

Breast; produces EGF receptor; overexpression

Front 153

ret

Back 153

MEN II & III, medullary thyroid CA; produces glial neurotrophic factor receptor; Point mutation

Front 154

abl

Back 154

CML, ALL; produces signal transduction proteins; translocation t(9:22), Philadelhpia chromosome

Front 155

Ki-ras

Back 155

Lung, pancreas and colon; produces GTP binding proteins; Point mutation

Front 156

c-myc

Back 156

Burkitt lymphoma; produces nuclear regulatory protein; translocation t(8:14). When associated to p53 apoptosis; when associated to bcl-2 inhibits apoptosis

Front 157

L-myc

Back 157

Small cell lung CA; produces nuclear regulatory protein; amplification

Front 158

N-myc

Back 158

Neuroblastoma; produces nuclear regulatory protein; amplification

Front 159

bcl-1

Back 159

Mantle cell lymphoma; produces cyclin D protein; translocation t(11:14)

Front 160

CDK4

Back 160

Melanoma; produces cdk; amplification

Front 161

bcl-2

Back 161

Normally prevents apoptosis. Follicular and undiferentiated lymphomas t(14:18). Chromosome 14: Ig heavy chain; chromosome 18 bcl-2

Front 162

c-kit

Back 162

gastrointestinal stromal tumor

Front 163

p53

Back 163

Normally prevents a cell with damaged DNA from entering S-phase by inhibiting cyclin/cdk. Colon, breast, CNS, lung. On chromosome 17

Front 164

Rb

Back 164

Normally prevents a cell from entering S phase. Retinoblastoma, osteogenic sarcoma. Inactivated product by cyclin/cdk. On chromosome 13

Front 165

Alpha fetoprotein

Back 165

Hepatocellular CA, testicular tumors

Front 166

B-hCG

Back 166

Trophoblastic tumors, chroriocarcinoma

Front 167

Calcitonin

Back 167

Medullary CA of thyroid

Front 168

CEA

Back 168

lung, pancreas, stomach, breast, colon

Front 169

CA-125

Back 169

Ovarian CA

Front 170

CA19-9

Back 170

Pancreatic CA

Front 171

Placental alkaline phosphatase

Back 171

Seminoma

Front 172

Prostatic acid phosphatase

Back 172

Prostate CA

Front 173

PSA

Back 173

Prostate CA

Front 174

Alkaline phosphatase

Back 174

Metastasis to bone, primary billiary cirrhosis

Front 175

VHL

Back 175

Chr 3p. Von Hippel Lindau, renal cell CA

Front 176

WT-1, WT-2

Back 176

11p. Wilm tumor

Front 177

BRCA-1

Back 177

17q. Hereditary breast and ovarian CA

Front 178

BRCA-2

Back 178

13q. Hereditary breast CA

Front 179

APC

Back 179

5q. Adenomatous polyps and colon CA

Front 180

DCC

Back 180

18q. Colon CA

Front 181

NF-1

Back 181

17q. Neurofibromatosis

Front 182

NF-2

Back 182

22q. Acoustic neuromas and meningiomas

Front 183

Down Syndrome

Back 183

ALL, AML

Front 184

Xeroderma pigmentosum, albinism

Back 184

Squamous cell, basal cell CA, melanoma

Front 185

Chronic atrophic gastritis, pernicious anemia, H. pylory

Back 185

Gastric adenocarcinoma

Front 186

Tuberous sclerosis

Back 186

Astrocytoma; produces PDGF; overexpression

Front 187

Actinic keratosis

Back 187

Squamous cell CA of skin and lung, angiosarcoma of liver

Front 188

Barret's esophagus

Back 188

Esophageal adenocarcinoma

Front 189

Plummer-Vinson syndrome

Back 189

Iron deficiency causes atrophic glositis, esophageal webs, anemia, squamous cell CA of esophagus

Front 190

Cirrhosis

Back 190

Hepatocellular CA

Front 191

Ulcerative colitis

Back 191

Colonic adenocarcinoma

Front 192

Paget's

Back 192

Osteosarcoma

Front 193

Immunodeficiency

Back 193

Malignant lymphomas

Front 194

AIDS

Back 194

Non-Hodgkin's, Kaposi

Front 195

Dysplastic nevus

Back 195

Malignant melanoma

Front 196

Radiation

Back 196

Sarcoma

Front 197

Small cell lung CA paraneoplastic syndromes

Back 197

Cushing's (ACTH), SIADH (ADH)

Front 198

Squamous cell lung CA paraneoplastic syndrome

Back 198

Hypercalcemia (PTH-like peptide), TGF-b, TNF, IL-1

Front 199

Renal cell CA paraneoplastic syndrome

Back 199

Polycythemia (EPO)

Front 200

Thymoma, Small cell lung CA paraneoplastic syndromes

Back 200

Lamber-Eaton (antibodies against presynaptic CA channels at neuromuscular junction)

Front 201

Leukemias and lymphomas paraneoplastic syndromes

Back 201

Gout (hyperuricemia due to excess nucelic acid turnover)

Front 202

Acanthosis nicrans

Back 202

Gastric adenocarcinoma

Front 203

Carcinoid tumor or medullary thyroid CA paraneoplastic syndromes

Back 203

Flushing, diarrhea (serotonin)

Front 204

alpha1-antitrypsin

Back 204

Hepatocellular CA

Front 205

Bence Jones protein

Back 205

Multiple Myeloma (Ig light chains in urine)

Front 206

MEN-I

Back 206

Pituitary (ACTH, Cushings), parathyroid (hypercalcemia), pancreas (zollinger-ellison, insulinoma)

Front 207

MEN-II

Back 207

medullary thyroid CA, parathyroid adenoma (hypercalcemia), pheocromocytoma (hypertension)

Front 208

5 leading causes of death in US

Back 208

1. Heart disease; 2. Cancer; 3. Cerebrovascular disease; 4. COPD; 5. Accidents

Front 209

3 leading causes of death in children in US

Back 209

1. Accidents; 2. Cancer; 3. Congenital abnormalities

Front 210

3 most common cancers in males

Back 210

1. Prostate; 2. Lung and bronchus; 3. Colon/rectum

Front 211

3 most common cancers in females

Back 211

1. Breast; 2. Lung; 3. Colon/rectum

Front 212

Top 3 cancer mortality in males

Back 212

1. Lung; 2. Prostate; 3. Colon/rectum

Front 213

Top 3 cancer mortality in females

Back 213

1. Lung; 2. Breast; 3. Colon/rectum

Front 214

5 causes of tissue hypoxia

Back 214

ischemia, hypoxemia, ETC block, uncoupled ETC, AV shunts

Front 215

Ultimate effects of tissue hypoxia

Back 215

No O2 to accept electrons in ETC, no production of ATP. Na/K pump fails and cell swells (reversible change). Ribosomes fall from RER. Disruption of cell membrane and mitochondria induces apoptosis.

Front 216

Effects of low ATP in cell

Back 216

Increased glycolysis to support ATPase pump. Anaerobic glycolysis produces lactate with decreased intracellular pH which denatures proteins (coagulation necrosis), cell swelling, entry of calcium and apoptosis

Front 217

Pathophysiology of cell injury in hypoxia

Back 217

ETC fails due to lack of oxygen; 2. No ATP production in ETC increases anaerobic glycolysis (high citrate and AMP activate PFK-1); 3. increased lactate decreases cell pH which denatures proteins and produces coagulation necrosis; 4. ATPase fails and cell swells with fall off of ribosomes from RER; 5. disruption of cell membrane with entry of Ca activates phspholipase (lipid peroxidation), complement activation, nuclear enzymes with pyknosis and destruction of mitochondria and apoptosis

Front 218

What is methhemoglobin?

Back 218

Hemoglobin with oxidized (Fe3+) iron that cant bind O2. Decreases SaO2 and produces cyanosis. Caused by nitro/sulfa compounds. Rx.: methylene blue

Front 219

Increased PACO2, decreased PaO2, decreased O2 content, decreased SaO2

Back 219

Respiratory acidosis

Front 220

Normal PaO2 and SaO2, decreased Hb

Back 220

Anemia

Front 221

Normal Hb, PaO2, decreased SaO2, decreased O2 content

Back 221

CO poisoning or methhemoglobinemia

Front 222

CO poisoning tissue hypoxia

Back 222

Decreased O2 content and SaO2, normal PaO2, left shift of dissociation curve and cytochrome oxidase inhibition all cause hypoxia. Produced by car exhaust, heaters, smoke inhalation, wood stoves. Rx.: 100% O2. First symptom: headache

Front 223

Factors that left-shift O2 dissociation curve and decrease P50

Back 223

Decreased 2,3BPG, CO, MetHb, HbF, hypothermia, alkalosis

Front 224

Factors that right-shift O2 dissociation curve and increase P50

Back 224

Increased 2,3BPG, fever, acidosis

Front 225

Causes of hypoxia with normal O2 content

Back 225

Ischemia, cyanide poisoning, ETC uncouplers (alcohol, salicylates, dinitrophenol)

Front 226

Free radical metabolism

Back 226

NADPH oxidase and spontaneous superoxide, Superoxide dismutase makes H2O2 from superoxide. Catalase breaks down H2O2. Gluthathione reductase and GSH peroxide breakdwon H2O2 using reduced GSH and NADPH from G6PDH in HMP shunt

Front 227

Causes of free radical injury

Back 227

Aging process produces lipofuscin which peroxidates membrane; MPO system, O2 free radicals, ionizing radiation, acetaminophen (treat with acetylcyteine), CCl4 poisoning

Front 228

Features of apoptosis

Back 228

Eosinophilic cytoplasm; pyknotic nucleus, no inflamatory infiltrate

Front 229

Physiologic examples of apoptosis

Back 229

Thymus involution, Mullerian and Wolffian structure involution, gravid uterus

Front 230

Pathologic examples of apoptosis

Back 230

Councilman bodies in viral hepatitis, psammoma bodies, cancer

Front 231

Coagulation necrosis

Back 231

Denaturing and coagulation of proteins in cytoplasm (infarction). Pale Vs. hemorrhagic infarcts

Front 232

Liquefactive necrosis

Back 232

Neutrophil destruction with hemolytic enzymes. Abesesses, wet gangrene, brain, pancreas

Front 233

Caseous necrosis

Back 233

Combination of coagulation and liquefaction necrosis. Cheese-like material, casseating granulomas with macrophages

Front 234

Fat necrosis

Back 234

Lipases on fatty tissue. Pancreas. Chalky-white appearance

Front 235

Fibrinoid necrosis

Back 235

Histologically resembles fibrin. Eosinophilic mitral valve vegetations, immunocomplexes

Front 236

Fatty liver change

Back 236

In alcoholics - liver stores excess triglycerides because increased NADH produces glycerol 3P and increased acetate (acetyl CoA) increases FA synthesis. In kwashiorkor, no apolipoproteins for VLDL

Front 237

Regulation of apoptosis

Back 237

Genes bcl-2 (inhibits apoptosis) prevents release of cytochrome C and binds protease activating factor (Apaf-1); p53 stimulates apoptosis. Mediated by caspases. Stimulated by cell injury, lack of hormones, Fas and TNF

Front 238

Rb suppressor gene and Rb protein

Back 238

Located on chromosome 13. Produces unphosphorylated Rb protein which stops cell from entering S phase. Phosphorylation by cyclin D/cdk complex allows it to enter S phase. Mutation of Rb gene produces cancer

Front 239

cdk/cyclin D complex

Back 239

When activated it phosphorylates Rb protein allowing cell to enter S phase

Front 240

p53 suppressor gene

Back 240

Located on chromosome 17. Produces a protein that inactivates cyclin D/cdk complex preventing Rb protein phosphorylation which keeps cell in G1

Front 241

Transient ischemic attack

Back 241

Reversible synptoms last less than 24 hours. Due to platelet thrombi or atheroemboli

Front 242

Infarction stroke

Back 242

85% of strokes. Can be thrombotic (atherosclerosis) white infarct or embolic (thromboemboli or atheroemboli) hemorrhagic infarct

Front 243

Clinical features of medial cerebral artery stroke

Back 243

Contralateral hemiparesis and sensory loss, aphasia if Broca area in the left hemisphere is involved, deviation of head and eyes toward side of lesion

Front 244

Types of hemorrhagic strokes

Back 244

Intraparenchymal, epidural, subdural, subarachnoid. Hemorrhagic strokes are 15% of all strokes

Front 245

Causes of hemorrhagic stroke

Back 245

Hypertension is MCC due to formation of microaneurysms that tend to rupture in basal ganglia or cerebellum. Arteriovenous malformations, amyloid, neoplasms, vasculitides

Front 246

Clinical features of hemorrhagic stroke

Back 246

Acute onset of severe headache, nausea, vomiting and coma

Front 247

Epidural hemorrhage

Back 247

Due to trauma and skull fracture with tear of middle meningeal dural artery. "Talk and die" syndrome. Leads to herniation if not promptly evacuated

Front 248

Subarachnoid hemorrhage

Back 248

MCC: ruptured berry aneurysm. Sudden thunderclap headache, nuchal rigidity, neurological deficits and coma

Front 249

Berry aneurysms

Back 249

Thin-walled saccular outpuchings consisting of intima and adventitia only. Cause of subarachnoid hemorrhage. Associated with Marfan, Ehlers-Danlos and adult polycystic kidney disease

Front 250

Spina bifida occulta

Back 250

Bony defect of vertebral arch

Front 251

Meningocele

Back 251

Bony defect with outpuching of the meninges

Front 252

Meningomyelocele

Back 252

Defective bony arch with outpuching of meninges, spinal cord and spinal roots. May lead to paraplegia and urinary incontinence

Front 253

Myelocele

Back 253

Defective bony arch with complete exposure of spinal cord. May lead to paraplegia and urinary incontinence

Front 254

Multiple sclerosis

Back 254

Chronic relapsing-remitting episodes of demyelination in brain and spinal cord with progressive neurological deficits. Blurred vision or loss of vision, diplopia and vertigo, loss of sensation or weakness in one leg, hemiparesis. TH1 cytokines (IF-g, TNF) facilitate attack; TH2 cytokines (IL-4, IL-10) retard attack.

Front 255

Parkinson disease clinical features

Back 255

Loss of dopaminergic neurons in the substantia nigra. Resting tremor, rigidity and akinesia, expressionless face. Rx.: levodopa

Front 256

Microscopic features of Parkinson disease

Back 256

Lewy bodies: intracytoplasmic round eosinophilic inclusions that contain alpha-synuclein; EM shows filaments of cytoskeletal origin

Front 257

Huntington disease clinical features

Back 257

Degeneration of GABAnergic neurons of caudate nucleus. Chrorea, dementia between ages 20-40. Triplet repeat CAG and features of genetic anticipation and imprinting

Front 258

Clinical features of Alzheimer disease

Back 258

MCC of dementia. Insidious inset of memory impariment, alterations in mood and behavior, aphasia and apraxia

Front 259

Microscopic features of Alzheimer disease

Back 259

Amyloid precursor protein (APP gene) on chromosome 21 (Down syndrome). AB amyloid from the APP protein. Senile plaques: AB amyloid tangled with neuritic processes, microglia and astrocytes. Neurfibrillary tangles: intraneuronal aggregates of paired helical filaments protein.

Front 260

Metyrapone stimulation test

Back 260

Metyrapone blocks 11OHase which stimulates ACTH and 11-deoxycortisol and decreases cortisol. If ACTH increases and 11-deoxycortisol decreases the problem is adrenal insuficiency. If both increase, the problem is pituitary insuficiency. If 11-deoxycortisol or ACTH dont change look for adrenal or ectopic Cushing's.

Front 261

High dose dexamethasone suppression test

Back 261

Dexamethasone is a cortisol analog and should suppress ACTH and cortisol. If it does suppress cortisol its pituitary Cushing's. If it doenst, its adrenal or ectopic Cushing's

Front 262

Clinical features of hyperthyroidism

Back 262

Tachychardia, palpitations, atrial fibrillation, systolic hypertension, nervousness, diaphoresis, tremors, diarrhea, weight loss. High free T4 and decreased TSH (if primary) or increased TSH (if secondary)

Front 263

Graves disease

Back 263

IgG autoantibodies stimulate TSH receptors. Signs and symptoms of hyperthyroidism plus goiter, exophthalmus and pretibial myxedema (due to glycosamynoglycan deposition)

Front 264

Effect of oral contraceptives and anabolic steroids on binding proteins

Back 264

Contraceptives increase binding proteins and increase total levels of the hormone with normal TSH or ACTH. Anabolics do the opposite. Increased total T4 or total cortisol with normal TSH or ACTH indicates contraceptives. Decreased total T4 or cortisol with normal TSH or ACTH indicates anabolic steroids.

Front 265

Clinical features of hypothyroidism

Back 265

Fatigue, cold sensitivity, decreased cardiac output, myxedema, constipation, anovulatory cycles. Low free T4 with high TSH (primary) or low TSH (secondary)

Front 266

Cretinism

Back 266

Congenital hypothyroidism. Mental retardation, failure to thrive, stunted bone growth.

Front 267

Hashimoto thyroiditis

Back 267

Anti-microsomal antibodies against thyroid gland produces hypothyroidism. Lymphocytic inflamation with germinal centers

Front 268

Subacute thyroiditis

Back 268

Preceded by viral illness. Granulomatous inflamation

Front 269

Reidel thyroiditis

Back 269

Destruction of the thyroid gland by dense fibrosis. Irregular hard thyroid that is adherent to trachea (dyspnea) and esopahgus (dysphagia)

Front 270

Thyroid adenoma

Back 270

Painless solitary cold nodule that may be functional

Front 271

Papillary thyroid carcinoma

Back 271

80% of malignant thyroid tumors. Papillary pattern, psammoma bodies, clear "orphan Annie" nuclei. Lymphatic spread to cervical nodes is common

Front 272

Follicular thyroid carcinoma

Back 272

15% of malignant thyroid tumors. Hematogenous metastasis to bone or lungs

Front 273

Medullary thyroid carcinoma

Back 273

5% of malignant thyroid tumors. Arises from C cells and produce calcitonin. May be associated with MEN II

Front 274

Anaplastic thyroid carcionoma

Back 274

Firm enlarging mass that metastasizes to trachea and esophagus and causes dyspnea and dysphagia

Front 275

Primary hyperparathyroidism

Back 275

Excess PTH with hypercalcemia caused by parathyroid adenoma (80%), hyperplasia (15%) or paraneoplastic syndromes (lung SCC and renal cell carcinoma). High serum Ca+ and PTH, kidney stones, osteoporosis, short QT interval.

Front 276

Secondary hyperparathyroidism

Back 276

Caused by chronic renal failure (no phosphate excretion increases serum phosphate, decreasing Ca+ and increasing PTH), chronic renal failure also causes deficiency of alpha-1-hydroxylase and vitamin D. Vitamin D deficiency and malabsorption

Front 277

Functions of PTH

Back 277

Increase reabsorption of Ca+ in distal tubule, decreases phosphate reabsorption in proximal tubule, increases alpha-1-hydroxylase in proximal tubule, binds to PTH receptor on osteoblast releasing IL-1 (osteoclast activating factor) to activate osteoclast resorption

Front 278

Hypoparathyroidism

Back 278

Due to surgical removal of glands or DiGeorge syndrome. Hypocalcemia with low PTH, tetany, prolonged QT interval

Front 279

Prolactinoma

Back 279

Hyperprolactinemia produces galactorrhea, amenorrhea (tumor mass inhibits GnRH) and infertility

Front 280

GH producing adenoma

Back 280

High GH and somatomedin C (IGF-1) produce gigantism (in children, tall stature with long extremities) or acromegaly (in adults, prominent jaw, flat forehead, enlarged hands and feet, diabetes and visceromegaly)

Front 281

Sheehan syndrome

Back 281

Ischemic necrosis of pituitary secondary to post partum hypotension results in panhypopituitarism

Front 282

Diabetes insipidus

Back 282

Hypotonic polyuria, polydipsia, hypernatremia, dehydration. Central DI is due to lack of ADH. Nephrogenic DI is due lack of renal response to ADH.

Front 283

SIADH

Back 283

Excesive ADH. Oliguria, water retention, hyponatremia, cerebral edema. Due to paraneoplastic syndrome (lung SCC) or trauma

Front 284

Clinical features of Cushing's syndrome

Back 284

Thin extremeties (protein catabolism), truncal obesity and buffalo hump (hyperglycemia increases insulin with fat deposition), purple stria (low collagen in vessels), hyperlipidemia (hormone-sensitive lipase), hirsutism, hypertension and hypokalemic alkalosis (high aldosterone).

Front 285

Differentiation of Cushing's syndrome

Back 285

High ACTH with dexamethasone suppression --> pituitary. High ACTH without dexamethasone suppression --> ectopic (lung SCC). Low ACTH --> adrenal. Low ACTH with low cortisol and adrenal atrophy --> steroid therapy (MCC). High ACTH produces skin pigmentation in pituitary and ectopic.

Front 286

Conn syndrome

Back 286

Adrenocortical adenoma causes hypertension, hypernatremia, hypokalemia, metabolic alkalosis, tetany. High aldosterone and low renin

Front 287

Waterhouse-Friderichsen syndrome

Back 287

Bilateral hemorrhagic infarction of the adrenals associated with menigococcemia. DIC, hypotension, shock.

Front 288

Addison disease

Back 288

Autoimmune destruction of adrenal cortex due to abrupt withdrawal of corticosteroids, miliary TB or meningoccemia. Weakness, hyperpigmentation of skin (high ACTH), hypotension, hypoglycemia, poor response to stress

Front 289

Pheochromocytoma

Back 289

Catecholamine-producing benign tumor of the adrenal medulla. Severe headaches, tachycardia, palpitationss, diaphoresis, anxiety, hypertension. Associated with MEN II. Elevated urinary vanillylmandelic acid (VMA) and catecholamines.

Front 290

MEN I

Back 290

Tumors of the pituitary (non-functioning), parathyroids (hypercalcemia), and pancreas gastrinoma (zollinger-ellison)

Front 291

MEN II

Back 291

Medullary carcinoma of thyroid, pheochromocytoma, parathyroid hyperplasia or adenoma

Front 292

Pathophysiology of diabetic ketoacidosis

Back 292

Hyperglycemia (due to increased glycogenolysis and gluconeogenesis). Ketone bodies (low insulin and high cortisol/epinephrine activate hormone-sensitive lipase, B-oxidation and ketogenesis). Osmotic diuresis and volume depletion with loss of potassium. Dilutional hyponatremia due to osmotic effect of hyperglycemia. Low insulin fails to activate lipoprotein lipase leading to hypertriglyceridemia.

Front 293

Pathophysiology of diabetic vascular disease

Back 293

Non-enzymatic glycosylation and arteriosclerosis produces ischemic injury and diabetic foot. Accelerated atherosclerosis, abdominal aortic aneurysms and MI.

Front 294

Pathophysiology of diabetic ocular disease

Back 294

Cataracts due to conversion of glucose into sorbitol by aldose reductase in the lens. Retinopathy with microaneurysms (non-proliferative) and neovascularization (proliferative)

Front 295

Pathophysiology of diabetic neuropathy

Back 295

Osmotic destruction of Schwann cells with decreased peripheral sensitivity leads to pressure ulcers on bottom of diabetic foot

Front 296

Features of tracheoesophageal fistula

Back 296

Blind esophagus is most common. Cyanosis, choking, vomit after feeding and polyhydramnios

Front 297

Plummer-Vinson syndrome

Back 297

Middle age women, esophageal webs with dysphagia to solids, iron deficiency anemia, increased risk of carcinoma

Front 298

Dysphagia to solids

Back 298

Esophageal webs in Plummer Vinson syndrome, obstruction due to cancer, Barret esophagus, mitral stenosis

Front 299

Dysphagia to solids and liquids

Back 299

No peristalsis. Miastenia gravis, CREST, achalasia

Front 300

Clinical features of achalasia

Back 300

Failure of LES to relax due to missing ganglion cells with lack of peristalsis. Dysphagia to solids and liquids, proximal esophageal dilation with bird-beak sign

Front 301

Mallory-Weiss syndrome

Back 301

Painful laceration at gastroesophageal junction due to severe prolonged vomiting in alcoholics or bulimia. Hematemesis. Complication: Boerhaave syndrome (esophageal rupture)

Front 302

Esophageal varices

Back 302

Dilated branches of left gastric vein secondary to portal hypertension in cirrhosis. Massive unpainful hematemesis.

Front 303

Reflux esophagitis

Back 303

Esophageal irritation to due reflux gastric secretions. Heartburn, regurgitation. Associated with Barret's esophagus

Front 304

Barret esophagus

Back 304

Squamous-columnar dysplasia of distal esophagus due to gastric reflux. Associated with adenocarcinoma

Front 305

Esophageal squamous cell carcinoma

Back 305

Most common in the world, second most common in US. Due to heavy smoking, alcohol, Plummer-Vinson. Progressive dysphagia to solids, bleeding, weightloss

Front 306

Esophageal adenocarcinoma

Back 306

Most common in US. In the distal esophagus. Associated with Barret esophagus dysplasia.

Front 307

Pyloric stenosis

Back 307

Non-bile projectile vomiting in the second week of life with palpable abdominal olive mass. Associated with Turner and Edwards.

Front 308

Menetier disease

Back 308

Hypertrophic rugal folds in body and fundus. Hyperplasia with replacement of parietal and chief cells with decreased acid production, protein loss due to lack of acid to activate pepsinogen and increased risk of gastric adenocarcinoma

Front 309

Zollinger-Ellison syndrome

Back 309

Pancreatic gastrin-producing gastrinoma. Hypertrophic rugal folds with increased acid secretion and multiple intractable peptic ulcers

Front 310

Acute hemorrhagic gastritis

Back 310

Inflamation and hemorrhage of gastric mucosa due to acid-induced mucosal breakdown. Epigastric abdominal pain, hemorrhage, hematemesis, melena. Caused by aspirin/NSAID (inhibits COX/PGE2), alcohol, smoking, postsurgery, burns.

Front 311

Stress ulcers

Back 311

Multiple small superficial ulcers caused by NSAIDs, severe stress, sepsis, shock

Front 312

Chronic gastritis type A

Back 312

Autoantibodies against parietal cells and/or intrinsic factor. Decreased acid secretion, increased serum gastrin (G-cell hyperplasia), pernicious anemia. Mucosal atrophy with rugal fold loss and chronic lymphoplasmacytic infiltrate. Increased risk of adenocarcinoma

Front 313

Chronic gastritis type B

Back 313

Urease-producing curved gram negative rod H. pylori visible with silver stain. Chronic inflamation with lymphoid follicles. Duodenal and gastric peptic ulcers associated with adenocarcnimoa

Front 314

Duodenal peptic ulcer

Back 314

Burning epigastric pain 1-3 hours after eating which is relieved by food. Associated with H. pylori (100%), increased gastric secretions. Anterior wall of proximal duodenum.

Front 315

Gastric peptic ulcer

Back 315

Burning epigastric pain which worsens with eating. Associated with H, pylori (75%). Small solitary punched-out ulcers in lesser curvature of the anthrum

Front 316

Etiology and clinical features of gastric carcinoma

Back 316

Asymptomatic until late in course. Epigastric abdominal pain, achlorydia, weight loss, ocult bleeding and iron deficiency anemia. Smoked fish and nitrosamines, H. pylori, chronic atrophic gastritis, smoking, Menetier.

Front 317

Microscopic features of gastric carcinoma

Back 317

Large irregular ulcer with necrotic base. Signet-ring cells (nucleous displaced to the periphery by intracellular mucin) and Linitis plastica (leather bottle-like stomach)

Front 318

Virchow (sentinel) node

Back 318

Left supraclavicular non-tender mass and epigastric distress due to gastric carcinoma metastasis

Front 319

Krukenberg tumor

Back 319

Gastric carcinoma metastasis to ovary with signet-ring cells on ovary

Front 320

Volvulus

Back 320

Twisting of bowelon its vascular mesentery resulting in obstruction and infarction

Front 321

Intussusception

Back 321

Telescoping of proximal into distal segment of bowel. Bloody stools, colic pain, infants.

Front 322

Hirchsprung disease

Back 322

Congenital absence of ganglion cells in Auerbach and Meissner plexuses of rectum and sigmoid colon. Delayed passage of mecomium, constipation, abdominal distention, vomiting, affected segment is narrow with proximal megacolon

Front 323

Celiac sprue

Back 323

Hypersensitivity to gluten with loss of small bowel villi and malabsorption. Abdominal distention, bloating and flatulence, steatorrhea. Associated with dermatitis herpetiformis

Front 324

Crohn disease

Back 324

Acolic pain with episodes of bloody diarrhea. Terminal ileum most common site. Discontinuous spread and ulcers with intervening normal mucosa, linear fissures, noncaseating granulomas, transmural inflammation and transmural sign on barium studies.

Front 325

Ulcerative colitis

Back 325

Continuous extensive ulcerations and pseudopolyps of rectum and colon with crypt abscesses in mucosa and submucosa. Associated with toxic megacolon and colon cancer. HLA-B27, arthritis, spondylitis

Front 326

Ischemic bowel disease

Back 326

Ischemia of the bowel secondary to atherosclerosis, thrombosis or shock. Abdominal pain and bloody diarrhea.

Front 327

Pseudomembranous colitis

Back 327

Inflammatory pseudomembranes in intestines due to C. difficile overgrowth secondary to clindamycin therapy.

Front 328

Appendicitis

Back 328

Periumbilical pain that subsequently localizes to the right lower quadrant with leukocytosis.

Front 329

Meckel diverticulum

Back 329

Asymptomatic congenital remnant of the vitelline (omphalomesenteric) duct. MCC of iron deficiency anemia in newborn.

Front 330

Colonic diverticulosis

Back 330

Out pouching and herniation of mucosa and submucosa through the muscularis propria. Most common in sigmoid colon. Left lower quadrant discomfort, occult bleeding and iron deficiency anemia. Complications: diverticulitis, fistulas

Front 331

Adenomatous colonic polyps

Back 331

Benign with adenocarcinoma potential. Commonly asymptomatic, occult bleeding with iron deficiency anemia. Sessile, villous is most malignant. Hemocult positive stools

Front 332

Bowel obstruction

Back 332

Colic pain, abdomen distention with no rebound tenderness, constipation, obstipation, step-ladder appearance on x-ray. Causes: adhesions from surgery (MCC), duodenal atresia, Hirchsprung.

Front 333

Familial adenomatous polyposis

Back 333

Autosomal dominant mutation on APC gene. Thousands of colonic adenomatous polyps. Invasive adenocarcinoma by age 40

Front 334

Gardner syndrome

Back 334

Variant of FAP with multiple osteomas, fibromatosis, epidermal inclusion cysts

Front 335

HNPCC

Back 335

Autosomal dominant mutation of DNA mismatch repair gene

Front 336

Colon adenocarcinoma

Back 336

Third most common in terms of incidence and mortaliy. Risk factors low-fiber diet, ulcerative colitis, FAP. Right colon presents with melena and iron deficiency anemia. Left colon presents with obstruction and reduced caliber stools

Front 337

Carcioind tumor

Back 337

Serotonin-producing tumor in appendix (MC) or terminal ileum produces metastasis to liver with subsequent heart carcinoid disease (tricuspid insufficiency, pulmonary stenosis). Diarrhea, flushing, bronchospasm, wheezing, fibrosis. Dx.: urinary 5-HIAA

Front 338

Clinical features of acute pancreatitis

Back 338

Stabbing epigastric abdominal pain radiating to the back, shock, hypocalcemia. Caused by gallstones, alcohol, hypercalcemia. Elevation of serum amylase and lipase

Front 339

Microscopic features of acute pancreatitis

Back 339

Liquefactive necrosis, acute inflammation, fat necrosis

Front 340

Complications of acute pancreatitis

Back 340

ARDS, DIC, pseudocyst, pancreatic calcifications

Front 341

Clinical features of chronic pancreatitis

Back 341

Abdominal pain, pancreatic insufficiency and malabsorption in middle age alcoholics. Chronic inflammation, atrophy and fibrosis.

Front 342

Clinical features of type I diabetes

Back 342

Polydipsia, polyuria, polyphagia, dehydration and electrolyte imbalance, metabolic ketoacidosis. Represents 10% of diabetes cases, associated to coxackie infection and autoimmune destruction of beta cells, HLA-DR3, DR4 and DQ

Front 343

Clinical features of type II diabetes

Back 343

90% of cases. Reduced insulin secretion and peripheral resistance to insulin. Polydipsia, polyuria, polyphagia.

Front 344

Vascular pathology in diabetics

Back 344

Major risk factor for atherosclerosis, MI, stroke. Atrophy of skin and loss of hair in lower extremities, claudication, nonhealing ulcers, gangrene, hyaline arteriosclerosis

Front 345

Diabetic nephropathy

Back 345

First sign is microalbuminuria. Hyaline arteriosclerosis of afferent and efferent arterioles. Diffuse nodular glomerulosclerosis and renal failure

Front 346

Diabetic retinopathy

Back 346

Nonproliferative phase: microaneurysms, retinal hemorrhage and exudates. Proliferative: neovascularization. Cataracts.

Front 347

Lab findings in type 2 diabetics

Back 347

Increased glucose, insulin and C peptide

Front 348

Lab findings in type 1 diabetics

Back 348

Increased glucose, low insulin and C peptide

Front 349

Lab findings in insulinoma

Back 349

Low glucose, high insulin and C peptide

Front 350

Lab findings in self-injection of insulin

Back 350

low glucose, high insulin, low C peptide

Front 351

Insulinoma

Back 351

Produces insulin. Hypoglycemia, elevated insulin and C-peptide. Rx.: glucose

Front 352

Gastrinoma

Back 352

Produces gastrin. Zollinger-Ellison: high gastrin, high gastric acid, intractable peptic ulcers

Front 353

Somatostinoma

Back 353

Produces somatostatin which inhibits insulin secretion (diabetes), gastrin secretion (hypochlorydia), cholecystokinin secretion (gallstones and steatorrhea)

Front 354

Pancreatic adenocarcinoma

Back 354

Abdominal pain, migratory thrombophlebitis, obstructive jaundice and clay colored stools. Most common site: pancreatic head. Tumor markers: CEA and CA19-9

Front 355

Clinical features of gallstones

Back 355

Right upper quadrant colic pain. Obesity -->cholesterol stones. Bilirubinate stones --> hemolytic anemias and cirrhosis. Complications: cholecystitis, choledocholithiasis, obstruction, pancreatitis.

Front 356

Acute cholecystitis

Back 356

Right upper quadrant colic pain, nausea, vomiting, fever, leukocytosis. Complications: gangrene, perforation, peritonitis.

Front 357

Ascending cholangitis

Back 357

Bacterial infection of bile ducts. Biliary colic, jaundice, high fever and chills

Front 358

Causes of unconjugated hyperbilirubinemias

Back 358

CB/total ration is < 20%. Hemolytic anemias, inneffective erythropoiesis, physiologic jaundice of newborn, Gilbert, Crigler-Najjar

Front 359

Gilbert syndrome

Back 359

Unconjugated bilirubinemia due to bilirubin glucoronosyltransferase (UGT) deficiency. Deficient conjugation. Jaundice is related to stress, fasting, infection.

Front 360

Crigler-Najjar syndrome

Back 360

Unconjugated bilirubinemia. Type 1 - fatal kernicterus, type 2 - jaundice. Glugoronosyltrasnterase absence or deficiency.

Front 361

Dubin-Johnson syndrome

Back 361

Conjugated bilirubinemia. Defect in canalicular transport with deficient excretion. Black pigmentation of liver.

Front 362

Causes of conjugated hyperbilirubinemias

Back 362

Dubin-Johnson, biliary tract obstruction by gallstones or tumors, PBC, primary sclerosing cholangitis

Front 363

Billiary tract obstruction

Back 363

By gallstones, pancreatic tumors. Conjugated bilirubinemia. Jaundice, pruritus, coluria, clay-colored stools, abdominal pain and fever

Front 364

Primary biliary cirrhosis

Back 364

Inflamation and granulomatous destruction of intrahepatic bile ducts. Middle age woman presents with obstructive jaundice, pruritus, xanthomas, xanthelasmas and high cholesterol. Anti-mitochondrial autoantibodies.

Front 365

Cirrhosis

Back 365

Fibrosis by Ito cells and regenerating nodules of hepatocytes. Due to alcohol, HBV, HCV, biliary tract disease, hemochromatosis. Leads to portal hypertension with ascites, splenomegaly, esophageal varices, hemorrhoids, hepatic encephalopathy, gynecomastia, hypoalbuminemia, low clotting factors, hepatorenal syndrome.

Front 366

Acute HBV markers

Back 366

HBsAg+, HBeAg+, HBcAb IgM+

Front 367

HBV window period markers

Back 367

HBcAb IgM+

Front 368

HBV prior infection markers

Back 368

HBcAb IgG+, HBsAb IgG+

Front 369

HBV immunization markers

Back 369

HBsAb IgG+

Front 370

HBV chronic infection markers

Back 370

HBsAg+, HBeAg+, HBcAb IgM+, HBcAb IgG+

Front 371

Alcoholic liver disease

Back 371

AST>ALT. Fatty change. In case of alcoholic hepatitis - RUQ pain, hepatomegaly, jaundice.

Front 372

Wilson disease

Back 372

Accumulation of copper due to decreased liver excretion. Fatty change, chronic hepatitis, micronodular cirrhosis, Kayser-Fleischer cornea rings, neurological manifestions. Low ceruloplasmin, high free copper, low total copper, high urine copper.

Front 373

Hemochromatosis

Back 373

High levels of iron due to increased absorption or secondary to transfusions. "Bronze diabetic", CHF. High ferritin and serum iron, low TIBC. HCC in 30%. Liver biopsy with Prussian blue stain.

Front 374

Budd-Chiari

Back 374

Hepatic vein thrombosis caused by polycythemia, contraceptives, pregnancy. Abdominal pain, hepatomegaly, ascites.

Front 375

HCC

Back 375

Due to cirrhosis, HBV, HCV, alcohol, hemochromatosis. Tumor marker - alpha-fetoprotein.

Front 376

Target cells

Back 376

increased RBC membrane. Hemoglobinopathies, thalassemia, liver disease.

Front 377

Acanthocytes

Back 377

Irregular spicules on surface. Abetalipoproteinemia

Front 378

Spherocytes

Back 378

Decreased RBC membrane. No central area of pallor. Spherocytosis

Front 379

Schistocytes

Back 379

RBC fragments. Microangiopathic hemolytic anemia, trauma

Front 380

Bite cells

Back 380

RBC with removed bits of cytoplasm. G6PDH deficiency.

Front 381

Sickle cells

Back 381

Sickle cell anemia

Front 382

Howell-Jolly bodies

Back 382

Remnants of nuclear chromatin. Severe anemias or patients without spleen

Front 383

Ring sideroblasts

Back 383

Trapped iron in mitochondria. Prussian-blue stain. Sideroblastic anemia

Front 384

Heinz bodies

Back 384

Denatured Hb. G6PDH deficiency

Front 385

Basophilic stipling

Back 385

RNA remnants. Lead poisoning

Front 386

Hypersegmented neutrophil

Back 386

Megaloblastic anemia

Front 387

EPO stimuli

Back 387

Low SaO2 (hypoxemia, anemia < 7gm/Dl, left shifted O2 curve

Front 388

Reticulocytes

Back 388

Immature RBC with no nucleous and bluish color in peripheral blood indicate effective erithropoiesis. Require 24 hours to become mature.

Front 389

Reticulocyte normal and corrected count

Back 389

Normal reticulocyte count is 1.5%. Corrected count is Hct/45 * reticulocyte count. >3% --> marrow responds well. <3% marrow is not well. If polychromasia (shift cells) divide corrected count by two because shift cells take double the time to mature

Front 390

Signs of anemia

Back 390

Palpitations, dizziness, angina, pallor, weakness

Front 391

Hypochromic RBCs

Back 391

Increased central pallor

Front 392

MCV < 80

Back 392

Iron deficiency, thalassemia, AOCD, Sideroblastic

Front 393

MCV 80-100, low reticulocyte count

Back 393

Marrow failure, aplastic anemia, leukemia, renal failure, AOD

Front 394

MCV 80-100, high reticulocyte count

Back 394

Sickle cell, G6PDH deficiency, spherocytosis, AIHA, PNH

Front 395

MCV > 100

Back 395

Folate or B12 deficiency

Front 396

Causes of iron deficiency anemia

Back 396

Ulcers, menstrual bleeding, left colon cancer, elderly and poor children, malabsorption, gastrectomy, hookworm, Plummer-Vinson

Front 397

Low serum iron, % saturation and serum ferritin with high TIBC

Back 397

Iron deficiency anemia

Front 398

Low serum iron, TIBC and % saturation with high serum ferritin

Back 398

AOCD

Front 399

High serum iron, serum ferritin and % saturation with low TIBC

Back 399

Sideroblastic anemia

Front 400

AOCD

Back 400

Iron is trapped in bone marrow macrophages due to high levels of IL-1 and lactoferrin. High ferritin and low TIBC.

Front 401

HbA

Back 401

α2β2

Front 402

HbF

Back 402

α2γ2

Front 403

Hb Barts

Back 403

γ4

Front 404

HbH

Back 404

β4

Front 405

α-thalassemia

Back 405

Carrier has one α gene deletion, asymptomatic. Α-Thal trait has two deletions. HbH disease three deletions with high HbH and Heinz bodies. Hydrops fetalis, four deletions, lethal, high Hb Barts

Front 406

β-thalassemia

Back 406

Minor, asymptomatic, 8% HbA2 and 5% HbF. Major - develop symptoms 6 months after birth as HbF declines, jaundice, bilirubin gallstones, secondary hemochromatosis due to life-long transfusions, CHF, crecut skull x-ray, target cells. 90% HbF and HbA2

Front 407

HbA2

Back 407

α2δ2

Front 408

Lead poisoning anemia

Back 408

Sideroblastic anemia. Lead denatures ferrochelatase, ALA dehydrse and ribonuclease (coarse basophilic stipling). Ringed sideroblasts and basophilic stipling. Lead colic, peripheral neuropahty, cerebral edema, learning disabilities, bone in epiphysis on x-rays. high serum Pb, high urine δ-ALA, high serum iron, ferritin and %saturation with low TIBC. Risk fators: Pb paint, battery factory, pottery painter.

Front 409

Iron overload anemia

Back 409

Sideroblastic anemia with ringed sideroblasts. Alcoholism (MCC), pyridoxine deficiency (required by ALA synthase), isoniazid treatment. High serum iron, % saturation, ferritin and decreased TIBC.

Front 410

Factors that induce and prevent sickling

Back 410

Deoxygenation of Hb/right shifting dissociation curve (acidosis), increasing HbS concentration (dehydration), low O2 tension (altitude and renal medulla). HbF left shifts dissociation curve and prevent sickling (hydroxeurea Rx)

Front 411

Pathophysiology of sickle cell disease

Back 411

Valine subsitutes glutamic acid in position 6 of β Hb chain causing sickling and thrombi that occlude vessels (painful crisis), hand-foot swelling, autosplenectomy with Howell-Jolly bodies and increased risk of infections by encapsulated orgainsms, Salmonella osteomyelitis, parvovirus B19 aplastic crisis.

Front 412

Pathophysiology of G6PDH deficiency

Back 412

Mutation causes defective protein folding with low G6PDH activity and low levels of reduced gluthathione needed to neutralize ROS. Oxidative stress, oxidative drugs (primaquine, sulfonamides, anti-TB), bacterial infections and fava beans cause red cell damage and hemolysis with Heinz body formation (seen with methylene blue or crystal violet stains)

Front 413

Pathophysiology of spherocytosis

Back 413

Spectrin defect with decrease in RBC membrane leads to circular RBCs which are removed by macrophages in the spleen (extravascular hemolysis). Triad of anemia, splenomegaly and jaundice with risk of bilirubinate gallstones. Increased osmotic fragility test.

Front 414

Pathophysiology of AIHA

Back 414

IgG autoantibodies against Rh antigens on RBC with macrophage removal in spleen cause splenomegaly. Differentiate from hereditary spherocytosis with positive direct Coombs test

Front 415

Pathophysiology of PNH

Back 415

Low levels of decay accelerating factor (DAF) are not able to normally inhibit C3 convertase with increased sensitivity of cells to complement lysis. Slow breathing at night (retains CO2) and exercise produce acidosis which activates the complement system with pancytopenia and increased risk of aplastic anemia, leukemia and venous thrombosis

Front 416

Direct Coomb's test

Back 416

Detects IgG or C3 on surface of RBCs. Positive in AIHA, negative in hereditary spherocytosis.

Front 417

Indirect Coomb's test

Back 417

Detects autoantibodies in the serum. Often positive in AIHA

Front 418

Pathophysiology of microangiopathic hemolytic anemia

Back 418

RBCs are damaged by calcium in stenotic valves (aortic stenosis MCC), fibrin clots in DIC and platelet plugs in ITP and HUS. Presence of schistocytes.

Front 419

Sites for reabsorption of iron, folate and B12

Back 419

Iron: duodenum (Bilroth II, vitamin c deficiency and malabsorption syndromes produce deficiency). Folate: jejunum (contraceptives and alcohol decrease absorption). B12: terminal ileum (pernicious anemia, Crohn's and terminal ileum resection decrease absorption)

Front 420

Pathophysiology of megaloblastic anemia

Back 420

Methyl THF is needed to make methylcobalamine to convert homocysteine into methione by methylTHF-homocysteine methyl transferase (requires cobalamine). Methylene THF is required by thymidilate synthetase to make nucleic acids. B12 is needed by methylmalonyl CoA mutase to make succinyl CoA.Tetrahydrofolate is made by dihydrofolate reductase (blocked by methotrexate and trimethropin). Deficiency of folate or B12 produces megaloblastic anemia with hypersegmented neutrophils (no nucleic acid synthesis), homocystinuria and methylmalonic aciduria.

Front 421

Causes of folate deficiency

Back 421

Alcoholism (not beer), pregnancy, methotrexate, trimetrhoprim, phentoyn, birth control pills, celiac disease, leukemia

Front 422

Causes of B12 deficiency

Back 422

Pernicious anemia, pure vegan diet, Crohn's disease, chronic pancreatitis (cant cleave R factor from saliva which protects B12), D. latum

Front 423

Schilling's test

Back 423

Non-radioactive intramuscular B12 to saturate transcobalamin followed by radioactivee oral B12. No radioactive B12 detected in 24h urine confirms B12 absorption deficiency. Correct with intrinsic factor (pernicious anemia), pancreatic enzymes (chronic pancreatitis) or antibiotics (bacterial overgrowth)

Front 424

Heterophile+ infectious mononucleosis

Back 424

EBV invades B lymphocytes via CD21 receptors with atypical CD8 response, lymphocytosis and paracortex hyperplasia. Fever, sore throat (gray-white membrane on tonsils) and tender lymphadenopathy

Front 425

Heterophile- infectious mononucleosis

Back 425

Cytomegalovirus

Front 426

Paul-Bunnell monospot test reaction

Back 426

IgM (heterophile) antibodies against EBV react with sheep red blood cells - postivie monospot test

Front 427

Characteristics of acute lymphadenopathy

Back 427

Tender focal lymphadenopathy = bacterial. Generalized tender lymphadenopathy = viral

Front 428

Characteristics of chronic lymphadenopathy

Back 428

Non-tender follicular hyperplasia (rheumatoid arthritis, toxoplasmosis, leukemia). Non-tender paracortical hyperplasia (viruses, drugs, SLE, leukemia).

Front 429

Leukemoid reaction Vs. leukemia

Back 429

Leukemoid reaction lacks blast and leukocyte alkaline phosphatase (LAP) (TB, whooping cough). Chronic myelogenous leukemia has low LAP.

Front 430

General signs and symptoms of leukemia

Back 430

Normo anemia, thrombocytopenia, leukocytosis or leukopenia, blast cells (>30%=acute), generalized non-tender lymphadenopathy, hepatosplenomegaly, bone pain and fever

Front 431

Pre-B ALL

Back 431

Age < 15. Tdt+, CALLA+, cytoplasmic mu+

Front 432

Mature B ALL

Back 432

Age < 15. Surface Igs present

Front 433

B cell CLL

Back 433

Age > 60. 95% of CLL cases. Differentiated cells are CD19+, CD20+, CD23+, CALLA-

Front 434

T cell CLL

Back 434

Age > 60. Mature T cell markers and hypogammaglubulinemia. Lymphocytosis and neutropenia

Front 435

Adult T cell leukemia

Back 435

Caused by HTLV-1 retrovirus. Leukemia sypmtoms and signs wih lytic bone lessions and hypercalcemia (osteoclast activating factor)

Front 436

AML

Back 436

15-60 years. Myeloblast proliferation. Auer rods are pathognomonic of myeloblasts. T(15;17). Abnormal retinoic acid receptor. Rx.: retinoic acid

Front 437

CML

Back 437

15-60 years. Pluripotent cell proliferation. Philadelphia chromosome t(9;22). All cells increased with low LAP

Front 438

PRV

Back 438

Increased erythroid precursors, hematocrit and viscocity. Decreased EPO. Normal SaO2. Increased basophils with histamine release (pruritus, gastric ulcers), plethora and cyanosis.

Front 439

Follicular B-cell lymphoma

Back 439

MC lymphoma. B lymphocytes. t(14;18), Chr 14 has immunoglobulin heavy chain genes, chr 18 has bcl-2 gene (normally inhibits apoptosis).

Front 440

Burkitt's lymphoma

Back 440

MC lymphoma in children. Starry-sky. t(8;14). African affects mandible, american affects abdomen

Front 441

Mycosis fungoides

Back 441

CD4 T-cells. Generalized prutitic erythematous rash. PAS+

Front 442

Histiocytosis X

Back 442

In children. Histiocytes are CD1+

Front 443

Hodgkin lymphoma

Back 443

Reed-Sternberg cells are CD15+, CD30+. Fever, night sweats, weight loss, localized lymphadenopathy

Front 444

Multiple myeloma

Back 444

Neoplasm of plasma cells. Anemia, bone pain, pathologic fractures, hypercalcemia, renal failure, light-chain amyloids (Bence-Jones protein).

Front 445

t(15;17)

Back 445

AML translocation

Front 446

t(9;22)

Back 446

CML philadelphia chromosome translocation. Forms a protein with tyrosine kinase activity

Front 447

t(14;18)

Back 447

Follicular B-cell lymphoma translocation

Front 448

t(8;14)

Back 448

Burkitt's lymphoma translocation

Front 449

Composition of bone

Back 449

Organic matrix: osteoblasts, osteoclasts, type I collagen, glycoproteins. Inorganic matrix: calcium hydroxyapatite, magnesium, potassium, chloride, sodium

Front 450

Osteoblasts

Back 450

Production of unmineralized bone. High amounts of alkaline phosphatase and PTH receptors to modulate osteoclasts

Front 451

Osteoclasts

Back 451

Bone resorption

Front 452

Achodroplasia

Back 452

Inherited dwarfism. Mutation on fibroblast growth factor receptor 3 inhibits cartilage synthesis at the epiphysial plates with decreased endochondral bone formation and premature ossification of growth plates. Short and thick extremities, large head and trunk.

Front 453

Osteogenesis imperfecta

Back 453

Abnormal synthesis of type I collagen. Recurrent fractures and deformities, thin blue sclera, deafness, thin skin and easy bruising

Front 454

Causes of osteoporosis

Back 454

Primary causes: estrogen deficiency with high IL-1 activity (postmenopausal, Turner's), genetic factors, lack of exercise, old age, nutritional. Secondary causes: Immobilization, Cushing's, thyrotoxicosis, malnutrition, corticosteroids.

Front 455

Clinical features of osteoporosis

Back 455

Bone is normal but in decreased amounts. Bone pain, compression fractures (vertebrae), femoral neck fracture, distal radius fracture.

Front 456

Radiographic, lab and microscopic features of osteoporosis

Back 456

Radiolucency of bone (osteopenia). Normal serum calcium, phosphorus and alkaline phosphatase. Thinned cortical and trabecular bone

Front 457

Treatment of osteoporosis

Back 457

Estrogen replacement therapy, weight bearing exercise, calcium, vitamin D, biphosphonates, calcitonin

Front 458

Causes of osteomalacia and rickets

Back 458

Decreased mineralization of newly formed bone due to abnormal metabolism of vitamin D. Dietary deficiency, intestinal malabsorption, lack of sunlight, renal and liver failure

Front 459

Clinical features of rickets

Back 459

Occurs in children. Craniotabes and frontal bossing (skull deformities), deformity of chest wall, pigeon breast, bowing of the legs. Low serum calcium with high alkaline phosphatase and PTH

Front 460

Clinical features of osteomalacia

Back 460

In adults, bone pain, fractures of vertebrae, hips and wrist. Low serum calcium, high alkaline phosphatase and PTH

Front 461

MC pathogen in osteomyelitis

Back 461

Staph

Front 462

Osteomyelitis in sickle cell disease

Back 462

Salmonella

Front 463

Osteomyelitis in drug abusers and diabetics

Back 463

Pseudomonas

Front 464

Clinical features of osteomyelitis

Back 464

Fever, leukocytosis, localized pain, erythema and swelling

Front 465

Clinical features of osteoarthritis

Back 465

Wear and tear joint chondrocyte injury with loss of articular collagen in weight-bearing joints. Stiffness, decreased range of motion, crepitus, pain that worsens with motion, old age. X-ray shows narrowing of joint space and osteophytes.

Front 466

Clinical features of rheumatoid arthritis

Back 466

Systemic, chronic inflamatory disease with anti-IgG autoantibodies (rheumatoid factor). Affects hands, wrists and knee, symetrical involvement, morning stiffness that improves with activity. Systemic manifestations: fever, fatigue, lymphadenopathy, rheumatoid subcutaneous skin nodules, Sjogren syndrome (15%)

Front 467

Microscopic, x-ray and lab findings in rheumatoid arthritis

Back 467

X-ray: juxta-articular osteoporosis and bone erosions. Micro: Synovitis, pannus (proliferation of synovium and granulation tissue over joint cartilage), radial deviation of wrist, ulnar deviation of fingers. Lab: high ESR, hypergammaglobulinemia, rheumatoid factor (anti-IgG).

Front 468

Ankylosing spondylitis associations

Back 468

young males, HLA-B27

Front 469

Reiter syndrome

Back 469

Conjunctivitis, chlamydia urethritis, arthritis of ankles and knees, HLA-B27, follows gonococus or chlamydia infection

Front 470

Causes of gout

Back 470

Primary (90%) idiopathic. Secondary: Leukemia, renal disease, Lesch-Nyhan, Von Gierke

Front 471

Clinical features of gout

Back 471

Exquisite pain in big toe. Negatively birefringent, needle-shaped monosodium urate crystals and neutrophils in aspiration. Complications: joint destruction, uric acid renal calculi, renal failure. Rx.: NSAIDs, colchicine, probenecid allopurinol

Front 472

Infectious arthritis clinical features

Back 472

Gonococci MC. Tender, swollen and erythematous joints, monoarticular arthritis. Cloudy sinovial fluid that clots, neutrophils and positive gram stain in aspiration

Front 473

Clinical features of myasthenia gravis

Back 473

Autoantibodies against Ach receptors at neuromuscular junction. Ptosis, diplopia, weakness. Associated with thymic hyperplasia and thymomas. Rx.: anticholinesterase agents, thymectomy

Front 474

Eaton-Lambert syndrome

Back 474

Paraneoplastic syndrome of SCC of lung. Autoantibodies against the presynaptic Ca+ channels at neuromuscular junction.

Front 475

Duchenne muscular dystrophy

Back 475

Absence of dystrophin muscle structural protein. Onset of symptoms by age 5, progressive muscular weakness, heart failure, respiratory insufficiency and infections. High serum CK.

Front 476

Guillain-Barre syndrome

Back 476

Preceded by viral illness. Ascending paralysis, loss of deep tendon reflexes. Inflamation and demyelination of peripheral nerves and spinal nerve roots result in muscular weakness

Front 477

Vitiligo

Back 477

Irregular, completely depigmented patches devoid of melanocytes.

Front 478

Melasma

Back 478

Irregular blotchy patches of hyperpigmentation on the face

Front 479

Freckles

Back 479

Light brown macules on face, shoulders and chest. Increased melanin deposition in the basal layer of the epidermis

Front 480

Nevocellular nevus (mole)

Back 480

Benign tumor of melanocytes with sharp well-circumscribed borders

Front 481

Malignant melanoma

Back 481

Asymetric, irregular borders, variegated color, large diameter, enlarging, macule, papule or nodule. Risk factors: chronic sun exposure, fair skin, dysplastic nevus syndrome

Front 482

Acanthosis nigricans

Back 482

Thickened, hyperpigmented skin in axillae and groin. Associated with malignancy and gastric adenocarcinoma

Front 483

Psoriasis

Back 483

Well demarcated erythematous plaque with a silvery scale. Epidermal hyperplasia (acanthosis), patchy keratinization with parakeratosis. Rx.: topical steroids and radiation

Front 484

Pemphigus

Back 484

Autoantibodies against desmosome component desmoglein 3 results in loss of intracellular adhesion (acantholysis) and blister formation. Easily ruptured, flaccid blisters. Immunofluorescence shows net-like pattern of IgG stainning

Front 485

Bullous pemphigoid

Back 485

Autoantibodies against hemidesmosome component bullous pemphigoid antigens 1 and 2 result in separation of epidermis from dermis and blister formation. Tense bullae that do not rupture easily. Eosinophils are seen

Front 486

Dermatitis herpetiformis

Back 486

IgA autoantibodies against gliadin that deposit in the tips of the dermal papillae with subepidermal clister formation. Microabsecesses with neutrophils are seen

Front 487

Squamous cell carcinoma of the skin risk factors

Back 487

Chronic sun exposure, fair complexion, chronic skin ulcers, xeroderma pigmentosa, actinic keratosis

Front 488

Squamous cell carcinoma of the skin clinical features

Back 488

Tan nodular mass which ulcerates on sun exposed areas. Micro: nests of atypical keratinocytes invade the dermis, formation of keratin pearls, desmosomes between tumor cells

Front 489

Basal cell carcinoma of the skin clinical features

Back 489

Chronic sun exposure, fair complexion, xeroderma pigmontosum. Pearly papules and nodules with heaped up translucent borders, telangiectasia, ulcerations. Palisading growth pattern

Front 490

Adult polycystic kidney disease

Back 490

Asymptomatic until middle age. Renal insuficiency, hematuria, hypertension. Abdominal masses and flank pain. Renal failure. Associated with liver cysts, berry aneurysms, mitral prolapse.

Front 491

Nephritic syndrome

Back 491

Hematuria, hypertension, azotemia, oliguria, proteinuria <3.5g/day

Front 492

Nephrotic syndrome

Back 492

Proteinuria >3.5g/day, hypoalbuminemia, generalized edema, hyperlipidemia

Front 493

Acute poststreptococal glomerulonephritis light microscopy

Back 493

Hypercellular glomeruli with neutrophils, red cell casts in renal tubules

Front 494

Acute poststreptococal glomerulonephritis immunofluorescence

Back 494

Granular deposits of IgG, IgM and C3 throughout the glomerulus

Front 495

Acute poststreptococoal glomerulonephritis electron microscopy

Back 495

subepithelial humps immune complex deposits

Front 496

Pathogenesis of Goodpasture syndrome

Back 496

anti-GBM antibodies damage kidneys and lungs. Antigen is collagen type IV

Front 497

Clinical presentation of Goodpasture syndrome

Back 497

nephritic syndrome with hemoptysis. Most will develop RPGN

Front 498

Goodpasture syndrome electron microscopy

Back 498

GBM disruption

Front 499

Goodpasture syndrome immunofluorescence

Back 499

Smooth and linear pattern of IgG and C3 in the GBM

Front 500

Causes of RPGN

Back 500

Goodpasture, poststreptococal, SLE, Wegner

Front 501

RPGN light microscopy

Back 501

Crescent formation in Bowman's space (macrophages, fibrin parietal endothelial cells)

Front 502

RPGN immunofluorescence

Back 502

granular or linear deposits of Ig and complement

Front 503

RPGN electron microscopy

Back 503

GBM disruption and discontinuity

Front 504

IgA nephropathy (Berger disease)

Back 504

Most common cause of GN. Nephritic syndrome with recurrent hematuria. Associated with celiac sprue and Henoch-Schonlein purpura

Front 505

IgA nephropathy light microscopy

Back 505

Mesangial proliferation

Front 506

IgA nephropathy immunofluorescence

Back 506

Mesangial deposits of IgA and C3

Front 507

IgA nephropathy electron microscopy

Back 507

Mesangial immune complex deposits

Front 508

MPGN clinical features

Back 508

Nephiritic or nephrotic. Decreased C3. C3 nephritic factor antibody activates C3 convertase with degradation of C3

Front 509

MPGN light microscopy

Back 509

Mesangial rpoliferation with BM thickening and tram tracking (splitting of basemant membrane)

Front 510

MPGN immunofluorescence

Back 510

Granular pattern of C3 often with IgG C1q and C4

Front 511

MPGN electron microscopy

Back 511

subendothelial and mesangial immune complex deposits

Front 512

Alport syndrome

Back 512

X-linked defect in type 4 collagen characterized ny nephritis, hearing loss and ocular abnormalities. EM: alternating thickening and thinning of BM

Front 513

Membranous GN etiology

Back 513

85% idiopathic, penicillamine, HBV, HCV, SLE, DM

Front 514

Membranous GN ligh microscopy

Back 514

Diffuse membrane-like thickening of capillary walls, baseman membrane projection spikes

Front 515

Membranous GN immunofluorescence

Back 515

Granular and linear pattern of IgG and C3

Front 516

Membranous GN EM

Back 516

Subepithelial deposits, effacement of podocyte foot processes

Front 517

Minimal change disease

Back 517

Nephrotic syndrome in children 2-6 years. EM: effacement of epithelial foot processes. Rx.: corticosteroids

Front 518

Focal segmental glomerulosclerosis light microscopy

Back 518

Focal segmental sclerosis and hyalinization of glomeruli

Front 519

Focal segmental glomerulosclerosis IF

Back 519

IgM and C3 deposits in sclerotic segments

Front 520

Mention all nephritic syndrome pathologies

Back 520

Poststreptococal, Goodpasture, IgA nephropathy, RPGN, MPGN, Alport

Front 521

Mention all nephrotic syndrome pathologies

Back 521

MGN, minimal changee disease, focal segmental glomerulonephritis

Front 522

Features of chronic glomerulonephritis

Back 522

Renal failure, uremia, anemia, proteinuria, hypertension, azotemia, reduction of GFR, hypocalcemia (no vitamin D), hyperphosphatemia. Hyalinization of glomeruli, fibrosis, atrophy and lymphocytes

Front 523

Features of acute tubular necrosis

Back 523

Reversible injury. Oliguria, increased BUN and creatinine, metabolic acidosis and hyperkalemia

Front 524

Causes of ischemic acute tubular necrosis

Back 524

hemorrhage, severe renal vasoconstriction, hypotension, dehydration, shock

Front 525

Causes of nephrotoxic acute tubular necrosis

Back 525

polymyxin, methicillin, gentamicin, sulfonamides

Front 526

Clinical features of acute pyelonephritis

Back 526

E. coli, proteus, kleibsiella, enterobacter. Fever, chills, dysuria, frequency, urgency, costovertebral angle tenderness, pyuria, WBC casts

Front 527

Types of renal calculi

Back 527

Calcium oxalate (75%), struvite (asoociated with urea-splitting bacteria - proteus), uric acid (gout, leukemia, acid urine)

Front 528

Clinical features of renal calculi

Back 528

Unilateral colic pain, hematuria, obstruction, infection. Calcium stones are raddiopaque.

Front 529

Clinical features of renal cell carcinoma

Back 529

Cigarette smoke is risk factor. Hematuria, palpable mass and flank pain

Front 530

Renal cell carcinoma paraneoplastic syndromes

Back 530

Polycythemia (EPO), hypertension (renin), Cushing syndrome

Front 531

Wilms tumor

Back 531

Mutations in WT-1 and WT-2 suppressor genes. WAGR syndrome - Wilm's tumor, aniridia, genital anomalies, mental retardation

Front 532

Condyloma acuminatum

Back 532

Verrucous wartlike lesions on vulva, perineum, vagina and cervix associated with HPV serotypes 6 and 11

Front 533

Pelvic inflammatory disease

Back 533

Vaginal discharge (cervicitis), vaginal bleeding (endometritis), bilateral lower abdominal and pelvic pain (salpingitis). Caused by n. gonorrhea and/or chlamydia. Complications: tubo-ovarian abscess, tubal scarring (granulomatous inflamation) with infertility and ectopic pregnancies

Front 534

Cervical cancer risk factors

Back 534

Early age of first intercourse, multiple sexual partners, multiple pregnancies, oral contraceptives, smoking

Front 535

Cervical cancer clinical features

Back 535

45 years old. Asymptomatic or postcoital bleeding, dyspareunia (painful intercourse), malodorous discharge. Caused by HPV types 16, 18, 31 and 33. Precursor lesion is cervical intraepithelial neoplasia

Front 536

Endometriosis

Back 536

Presence of endometrial glands and stroma outside the uterus in ovaries, ligaments and pouch of Douglas. Presents with chronic pelvic pain, dysmenorrhea, dyspareunia (painful intercourse), rectal pain, constipation, infertility

Front 537

Endometrial carcinoma risk factors

Back 537

Early menarche, late menapause, nulliparity, hypertension, diabetes, anovulation, estrogen-producing tumors, estrogen-replacement therapy, endometrial hyperplasia

Front 538

Endometrial carcinoma clinical features

Back 538

55 year old with postmenopausal vaginal bleeding

Front 539

Polycystic ovarian disease

Back 539

Females of reproductive age, oligomenorrhea, hirsutism, infertility. Lab: elevated LH, low FSH, high testosterone. Predisposes to endometrial cancer

Front 540

Ovarian cystadenocarcinoma

Back 540

65 year old with malignant bilateral ovarian enlargement. Risk factors: BRCA-1. Marker: CA125

Front 541

Metastatic tumors to the ovary primary sites

Back 541

Breast, colon, endometrial, gastric "signet-cell" Krukenberg tumor

Front 542

Hydatidiform mole

Back 542

Tumor of placental trophoblastic tissue/ Excessive uterine enlargement, vaginal bleeding, high B-HCG. Complete mole: fertilization of an ovum without chromosomes. Partial mole: fertilization by two sperms (one 23X, one 23Y)

Front 543

Choriocarcinoma

Back 543

Malignant germ cell tumor derived from the trophoblast

Front 544

Fibrocystic change presentation

Back 544

Bilateral painful mass in young woman with menstrual variation

Front 545

Fibroadenoma

Back 545

Movable mass that changes with mentrual cycles

Front 546

Breast carcinoma risk factors

Back 546

BRCA-1, BRCA-2, p53, prior breast cancer, old age, nulliparity, obesity

Front 547

Breast carcinoma clinical features

Back 547

Solitary painless mass in old woman with nipple retraction or skin dimpling or fixation to chest wall. Calcification on mamogram. MC variation is invasive ductal carcinoma

Front 548

Benign prostatic hyperplasia

Back 548

Decreased caliber and force of stream, urgency, frequency, nocturia, dysuria. PSA is elevated. Rx.: 5-alpha reductase inhibitor

Front 549

Prostate cancer

Back 549

Asymptomatic or lower back pain secondary to metastasis. High PSA. Metastasis to pelvic lymph nodes and lumbar spine with high alkaline phosphatase

Front 550

What will aspiration of a foreign body result in the lung?

Back 550

Obstruction atelectasis

Front 551

Presence of fluid, air or tumor in the pleural space results in what type of atelectasis?

Back 551

Compression atelectasis

Front 552

Contraction atelectasis is due to what cause?

Back 552

Fibrosis of the lung

Front 553

Causes of patchy atelectasis

Back 553

Lack of surfactant (hyaline membrane disease of newborn or ARDS)

Front 554

Clinical features of typical pneumonia

Back 554

Sudden onset, high fever, productive cough, tachypnea, pleuritic chest pain, consolidation on x-ray

Front 555

Clinical features of atypical pneumonia

Back 555

Insidious onset, low fever, no cough, no consolidation

Front 556

Differential diagnosis of rusty sputum

Back 556

Strep pneumonia, CHF, mitral stenosis, Goodpasture syndrome

Front 557

Features of sarcoidosis

Back 557

"GRAIN": gammaglubilinemia, rheumatoid arthritis, ACE increase, interstitial fibrosis, non-casseating granuloma, bilateral lymphadenopathy, hypercalcemia due to vitamin activation by macrophages

Front 558

Causes of restrictive pulmonary disease

Back 558

Kyphoscoliosis, obesity, pneumoconiosis, ARDS, pulmonary fibrosis, sarcoidosis

Front 559

Causes of obstructive pulmonary disease

Back 559

Asthma, emphysema, chronic bronchitis, bronchiectasis

Front 560

Lung volumes in obstructive pattern

Back 560

Increased TLC, FRC and RV. Decreased FEV1, FVC, FEV1/FVC

Front 561

Lung volumes in restrictive pattern

Back 561

Decreased, TLC, FEV1, FVC, FRC, RV. Increased or normal FEV1/FVC

Front 562

Diagnosis criteria for chronic bronchitis

Back 562

Persistent cough and copius sputum production for at least 3 months in 2 consecutive years

Front 563

Clinical features of chronic bronchitis

Back 563

Cough, sputum production, dyspnea, infections, hypoxia, cyanosis, weight gain. "Blue bloater"

Front 564

Microscopic findings in chronic bronchitis

Back 564

Hypertrophy of bronchial mucous glands, globlet cell hyperplasia, mucus hypersecretion, bronchial metaplasia

Front 565

Complications of chronic bronchitis

Back 565

Recurrent infections, cor pulmonale, lung cancer

Front 566

Definition of emphysema

Back 566

destruction of alveolar septa resulting in enlarged air spaces and loss of elastic recoil

Front 567

Etiology of emphysema

Back 567

Protease/antiprotease imbalance. Proteases are made by macrophages and neutrophils. Antiproteases are alpha-1-antitrypsin, alpha-1-macroglubulin and secretory leukoprotease inhibitor

Front 568

Features of centriacinar emphysema

Back 568

Proximal bronchioles involved, distal bronchioles spared, most common (95%), associated with smoking, worst in apical segments of upper lobes

Front 569

Features of panacinar emphysema

Back 569

Entire acinus invololved, alpha-1-antitrypsin deficincy, worse in bases of lower lobes

Front 570

Clinical features of emphysema

Back 570

Progressive dyspnea, pursing of lips and accesory muscles, barrel chest, weight loss, "Pink puffer"'

Front 571

Clinical features of asthma

Back 571

Wheezing, severe dyspnea, coughing

Front 572

Microscopic features of asthma

Back 572

Charcot-leyden crystals, mucous plugs, goblet cell hyperplasia and hypertrophy, eosinophils, edema, hypertrophy of smooth muscle, thick basement membranes

Front 573

Clinical features of bronchiectasis

Back 573

cough, fever, malodorous purulent sputum, dyspnea, dilated bronchi extending out to pleura on x-ray

Front 574

Etiology of bronchiectasis

Back 574

Bronchial obstruction, necrotizing pneumonia, cystic fibrosis, Kartagener syndrome

Front 575

Definition of acute respiratory distress syndrome

Back 575

damage of alveolar epithelium and capillaries resulting in respiratory failure that is unresponsive to O2 treatment

Front 576

Causes of ARDS

Back 576

shock, sepsis, trauma, gastric aspiration, radiation, O2 toxicity, drugs, infections

Front 577

Clinical features of ARDS

Back 577

dyspnea, tachypnea, hypoxemia, cyanosis, use of accesory respiratory muscles. Bilateral lung opacity on x-ray

Front 578

Microscopic features of ARDS

Back 578

interstitial and alveolar edema, interstitial inflamation, loss of type I pneumocytes, hyaline membrane formation

Front 579

RDS of newborn

Back 579

Deficiency of surfactant in prematures (<28 weeks) and sons of diabetic mothers. Dyspnea, tachypnea, nasal flaring and cyanosis. Lecithin:sphyngomyelin < 2. Rx.: surfactant and dexamethasone

Front 580

Causes of pulmonary edema

Back 580

left heart failure, mitral stenosis, fluid overload, nephrotic syndrome, liver disease

Front 581

Microscopic features of pulmonary edema

Back 581

Intra-alveolar fluid, engorged capillaries, hemosiderin-ladden macrophages

Front 582

Risk factors and genetics of bronchogenic CA

Back 582

Cigarette smoking, pneumoconiosis, pollution. Oncogenes: L-myc (SCC), K-ras (adeno). Suppressor genes: p53 and Rb

Front 583

Clinical features of bronchogenic CA

Back 583

Cough, sputum production, weight loss, anorexia, fatigue, dyspnea, hemoptysis, chest pain. Obstruction may produce emphysema, atelectasis, bronchiectasis or pneumonia

Front 584

Lung adenocarcinoma

Back 584

Most common - 35%. More common in women. Peripheral gray mass, scarring and mucin-producing glands

Front 585

Squamous cell carconima of lung

Back 585

2nd most common - 30%. More common in males, related to smoking. Centrally located. Invasive squamous cells with desmosomes and keratin production, PTH production

Front 586

Small cell carcinoma

Back 586

20%. More common in males, associated to smoking. Central location. Basophilic neurosecretory granules and paraneoplastic syndromes (ACTH, ADH)

Front 587

Pancoast tumor

Back 587

Apical tumor causing Horner syndrome (ptosis, miosis, anhidrosis, enopthalmos)

Front 588

Superior vena cava syndrome

Back 588

Obstruction, distended head and neck veins, plethora, facial edema

Front 589

Effects of lung masses within the thorax structures

Back 589

Pancoast tumor, superior vena cava syndrome, esopahgeal obstruction, recurrent laryngeal nerve hoarseness, Eaton-Lambert syndrome

Front 590

Sites of metastasis of lung cancer

Back 590

Adrenals (>50%), liver, brain, bone

Front 591

Eaton-lambert syndrome

Back 591

auto-antibodies against presynaptic Ca channels in neuromuscular junction

Front 592

Metastasis to the lung

Back 592

Breast (most common)

Front 593

Lab findings in PAN

Back 593

p-anca. HBsAg+ in 30%, anemia, leukocytosis

Front 594

Microscopic features of PAN

Back 594

Segmental necrotizing vasculitis in three stages: fibrinoid necrosis with neutrophils, fibroblast proliferation, nodular fibrosis with loss of internal elastic lamina

Front 595

Clinical features of PAN

Back 595

Affects all organs except lungs. Fever, hematuria/renal failure/hypertension, abdominal pain/GI bleeding, myalgia/arthralgia

Front 596

Clinical features of Wegner granulomatosis

Back 596

Bilateral pneumonitis with nodular and cavitary infiltrates, chronic sinusitis, nasopharyngeal ulcerations, renal disease

Front 597

Microscopic features of Wegner granulomatosis

Back 597

Necrotizing vasculitis of small vessels (granulomas), necrotizing granulomas of respiratory tract, focal necrotizing glomerulonephritis

Front 598

Lab findings in Wegner granulomatosis

Back 598

c-anca

Front 599

Treatment of Wegner granulomatosis

Back 599

cyclophosphimide

Front 600

Clinical features of temporal arteritis

Back 600

Throbbing unilateral headache, visual disturbances, jaw claudication

Front 601

Microscopic features of temporal arteritis

Back 601

Segmental granulomatous vasculitis with multinucleated giant cells and fragmentation of the internal elastic lamina with intimal fibrosis and luminal thickening

Front 602

Diagnosis, lab findings and treatment of temporal arteritis

Back 602

Dx.: biopsy of temporal artery. Lab: increased ESR. Rx.: steroids

Front 603

Clinical features of Takayasu asteritis

Back 603

Loss of pulse in upper extremities, visual disturbances, neurologic abnormalities

Front 604

Microscopic features of Takayasu arteritis

Back 604

Granulomatous vasculitis with massive intimal fibrosis, thickening of the aortic arch and narrowing of the major arterial branches

Front 605

Clinical features of Buerger's disease

Back 605

Severe pain in affected extremity, thrombophlebitis, Raynaud phenomenon, ulceration and gangrene. Associated with heavy cigarette smoking

Front 606

Microscopic features of Buerger's disease

Back 606

Recurrent neutrophilic vasculitis with microabseses, segmental thrombosis and vascular insuficiency

Front 607

Clinical features of Kawasaki disease

Back 607

Affects children < 4. Acute febrile illness, conjuctivitis, maculopapular rash, lymphadenopathy, coronary aneurysms in 70% of cases

Front 608

Microscopic features of Kawasaki disease

Back 608

Segmental necrotizing vasculitis with coronary aneurysms

Front 609

Diseases that feature Raynaud phenomenon

Back 609

SLE, CREST, Buerger, atherosclerosis

Front 610

Raynaud diseasse

Back 610

Small artery vasospasm resulting in blanching cyanosis of fingers and toes precipitated by cold temperature and emotions

Front 611

Henoch-Schonlein purpura

Back 611

IgA-C3 immunocomplexes, IgA nephropathy (Berger disease), palpable purpura on buttocks

Front 612

Major risk factors for atherosclerosis

Back 612

Hyperlipidemia, hypertension, smoking, diabetes

Front 613

Most common sites for atherosclerosis

Back 613

Abdominal aorta followed by coronary arteries

Front 614

Complications of atherosclerosis

Back 614

Ischemic heart disease, abdominal aortic aneurysm, peripheral vascular disease (pain, pulselessness, paresthesia, claudication), TIA (vertebral basilar oclussion), renovascular hypertension (high renin).

Front 615

Pathophysiology of essential hypertension

Back 615

Retention of sodium and water with increase in stroke volume (systolic pressure). Sodium in smooth muscle opens up calcium channels with vasoconstriction of arterioles (increased diastolic pressure). Low renin hypertension.

Front 616

Complications of hypertension

Back 616

Concentric ventricular hypertrophy, AMI, hyaline arteriosclerosis, nephrosclerosis and CRF, intracranial bleeds, atherosclerosis

Front 617

Renovascular hypertension

Back 617

Atherosclerosis of renal artery orifice in males or fibromuscular hyperplasia in women. Severe hypertension, epigastric bruit. High renin hypertension. Screen with captopril.

Front 618

Captopril screening test for renovascular hypertension

Back 618

In renovascular hypertension there's decreased RPF and high levels of renin and angiotensin II. With captopril (ACE inhibitor), there's loss of negative feedback on renin and exagerated high levels of renin post-stimulation. The test has the potential for renal failure if bilateral renal artery stenosis is present as AII is responsible for maintaining renal blood flow.

Front 619

Ahterosclerotic aneurysms

Back 619

MC site is abdominal aorta below renal arteries (no vasa vasorum). Pulsatile mass with pain and abdominal bruit

Front 620

Syphilitic aneurysm

Back 620

Obliterative endarteritis of vasa vasorum with ischemia and atrophy of ascending aorta, aortic insuficiency, airway encroachment and laryngeal nerve involvment (brassy cough)

Front 621

Associated diseases of dissecting aortic aneurysm

Back 621

Marfan, Ehlers-Danlos, copper deficiency (no lysyl oxidase)

Front 622

Signs and symptoms of dissecting aortic aneurysm

Back 622

Acute retrosternal severe chest pain, aortic insuficiency and cardiac tamponade

Front 623

Phlebothrombosis Vs. Thrombophlebitis

Back 623

Phlebothrombosis is venous thrombosis of deep veins without inflamation or infection. Thrombophlebitis is venous thrombosis of superficial veins due to inflamation and infection

Front 624

Signs, symptoms, diagnosis and complications of DVT

Back 624

Leg swelling, warmth, erythema. Increased venous pressure from deep to superficial veins (which drain in deep veins) produces varicosities in superficial system. Complications are thromboembolism, thrombophlebitis. Dx.: Doppler

Front 625

Signs, symptoms and causes of thrombophlebitis

Back 625

Palpable cord, pain, induration, warmth, erythema. MCC is superficial varicose veins, phlebothrombosis, catherthers, drug abuse

Front 626

Clinical features of varicose veins

Back 626

Edema, thrombosis, stasis dermatitis, ulcerations

Front 627

Clinical features of superior vena cava syndrome

Back 627

Compression of SVN by primary lung cancer. Blue discoloration of the face, arms and shoulders, dizziness, convulsions, visual disturbances, distended jugular veins

Front 628

Clinical features of Kaposi sarcoma

Back 628

Malignant endothelial cell tumor caused by HHV-8. Multiple red-purple patches, plaques or nodules. Spindle shaped cells

Front 629

Clinical features of stable angina

Back 629

Chest pain induced by exercise or emotions. ST segment depression (subendocardial ischemia) Relieved by rest or nitroglycerin

Front 630

Clinical features of Prinzmetal angina

Back 630

Chest pain caused by coronary artery vasospasm. ST segment elevation. Relieved by nitroglycerin

Front 631

Clinical features of unstable angina

Back 631

Non-occlusive thrombus triggers release of TXA2 (vasoconstrictor). Occurs at rest. Risk of MI

Front 632

Coronary irrigation of the heart

Back 632

Left anterior descending artery supplies anterior portion of left ventricle and anterior 2/3 of interventricular septum (produces heart blocks) (45% of MI). Circumflex artery, branch of left coronary artery (15% of MI). Right coronary artery supplies posterior and inferior left ventricle, right ventricle, SA node (sinus bradycardia), papillary muscle (mitral insuficiency) (35% of MI)

Front 633

Risk factors for coronary artery disease

Back 633

Age, family history, cigarette smoke, hipertension, low HDL, high LDL, diabetes

Front 634

Clinical presentation of AMI

Back 634

Sudden onset of acute substernal chest pain radiated to left arm, jaw and neck. Shortness of breath, diaphoresis, nausea, vomiting and anxiety

Front 635

Serum markers of miocardial infarction

Back 635

CK-MB elevated by 8h, peaks 18h, normal in 3 days. Troponin elevated by 6h, peaks 16h, normal in 10 days. LDH elevted by 24h, peaks 6 days, normal in 14 days.

Front 636

Gross changes in miocardial infarction

Back 636

18h, no change. 24h vague pallor. 1-7d yellow pallor. 7-28d central pallor with red border. Months - white firm scar

Front 637

Microscopic changes in miocardial infarction

Back 637

4-24h coagulative necrosis. 1-3d neutrophilic infiltrate. 3-7d macrophages. 7-28d granulation tissue. Months - fibrotic scar

Front 638

Complications of MI

Back 638

Arrhythmias (MC COD), CHF, pericarditis, rupture (4-7 days post-infarct). Ventricular free wall (LAD) --> cardiac tamponade. Interventricular septum (LAD) --> left to right shunt. Papillary muscle (RCA) mitral insufficiency

Front 639

Features of sudden cardiac death

Back 639

Death within 1 hour of onset of symptoms by fatal arrhythmia. CAD (80%), hypertrophic cardiomyopathy, mitral valve prolapse, aortic stenosis

Front 640

Pathophysiology of heart failure

Back 640

Left ventricle fails --> decreased cardiac output --> RAA system and retention of Na and H20 --> increased venous return causes edema and partial compensation of CO. There's backward pulmonary congestion that causes dyspnea and pulmonary edema with decreased RV output that adds up to systemic edema. Increased sympathetic tone and volume retention are compensation mechanisms

Front 641

Signs and symptoms of left heart failure

Back 641

Dyspnea (due to increased pulmonary hydrostatic pressure), pillow orthopnea (no gravity increases venous return with pulmonary congestion), rales, S3 gallop (volume overloaded ventricle)

Front 642

Complications of left heart failure

Back 642

Pulmonary edema, excessive RAA leads to secondary hyperaldosteronism, cardiogenic shock

Front 643

Features and treatment of systolic left heart failure

Back 643

Due to decreased contractility after infarction. EF<0.4. Rx. Inotropics (digitalis), decrease afterload with vasodilators (ACE inhibitor)

Front 644

Features and treatment of diastolic left heart failure

Back 644

Due to decreased compliance of left ventricle (increases left atrial pressure and pulmonary congestion). EF>0.4. Due to left ventricular hypertrophy, restrictive cardiomyopathy. Rx.: increase preload by decreasing heart rate (calcium channel blockers and B-blockers)

Front 645

Causes of right heart failure

Back 645

Left heart failure (MCC), cor pulmonale (primary pulmonary hypertension)

Front 646

Clinical features of right heart failure

Back 646

Jugular venous distension, nutmeg liver hepatomegaly, dependant pitting edema, ascites, pleural effusions, tricuspid insuficiency

Front 647

Causes and features of mitral stenosis

Back 647

Chronic rheumatic fever is MCC. Mid-diastolic murmur. Dyspnea and hemoptisis (pulmonary congestion), atrial fibrillation (left atrial dilation), dysphagia for solids (enlarged left atrium compresses esophagus), hoarseness (irritation of recurrent laryngeal nerve)

Front 648

Causes and features of mitral prolapse

Back 648

Valve leaflets undergo myxomatous degeneration. Associated with lethal ventricular arrhythmias in Marfan. Mid-systolic click. Infectious endocarditis and rupture of chordae tendinae are complications Rx.: CCA, b-blockers and negative inotropic agents

Front 649

Causes and features of mitral insuficiency

Back 649

Caused by mitral prolapse, left heart failure, infective endocarditis, RCA thrombosis (papillary muscle). Holosystolic murmur heard best at apex, S3 heart sound.

Front 650

Causes and features of aortic stenosis

Back 650

MCC is calcified congenital bicuspid valve, rheumatic fever, old age. Decreased stroke volume and cardiac output, increased afterload. Left ventricular hypertrophy. Systolic ejection murmur. Associated with angina (less coronary filling), syncope (reduced cardiac output) and microangiopathic hemolytic anemia with schistocytes

Front 651

Causes and features of aortic insuficiency

Back 651

MCC is essential hypertension, infective endocarditis, syphilitic and aortic aneurysms. Left ventricular hypertrophy, increased preload. Diastolic murmur, bounding pulse.

Front 652

Pathophysiology of rheumatic fever

Back 652

Antibodies against streptococal M protein cross react with heart valves producing fibrosis/stenosis, as well as systemic features

Front 653

Jones major criteria of rheumatic fever

Back 653

Migratory polyarthritis, pancarditis, subcutaneous nodules, erythema marginatum, sydenhan chrorea

Front 654

Pathognomonic lesion of rheumatic heart fever

Back 654

Aschoff body. Fibrinoid necrosis surrounded by macrophages (Anitschkow cells), lymphocytes and plasma cells

Front 655

Clinical features of subacute endocarditis

Back 655

Strep viridans colonizes damaged valves. "FROM JANE". Fever, Roth spots on retina, Osler nodes (painful subcutaneous nodules on fingers and toes), murmur, Janeway lesions (painless red lesions on palms and soles), anemia, nailbed hemorrhage, septic emboli

Front 656

Preductal coarctation of the aorta

Back 656

Associated with Turner syndrome. Narrowing of aorta proximal to ductus arteriosus. Ususally associated with PDA that supplies oxygenated blood to distal aorta. Presents in newborn with CHF, weak pulses and cyanosis of lower extremities.

Front 657

Postductal coarctation of the aorta

Back 657

Narrowing of aorta distal to ductus arteriosus. Hypertension in upper extremities and hypotension in lower extremities. Can produce aortic insuficiency, berry aneurysms and secondat hypertension due to increased RAA (low renal flow)

Front 658

Right to left shunts

Back 658

Early cyanosis due to blood shunt past the lungs. Tetralogy of Fallot, transposition of great vessels, truncus arteriosus, tricuspid atresia.

Front 659

Left to right shunts

Back 659

Late cyanosis due to Eisenmenger syndrome. VSD, ASD, PDA

Front 660

Eisenmenger syndrome

Back 660

Right side of the heart hypertrophies due to a septal defect or PDA and shunt reverses from left-right to right-left producing cyanosis

Front 661

Tetralogy of Fallot

Back 661

Overriding aorta, pulmonic stenosis, right ventricular hypertrophy, VSD. Cyanosis depends on degree of pulmonic stenosis. PDA or ASD are cardioprotective.

Front 662

Transposition of the great vessels

Back 662

Inversion of aorta and pulmonary arteries. Infants of diabetic mothers. Must have ASD, VSD or PDA to survive.

Front 663

Truncus arteriosus

Back 663

Common pulmonary artery and aortic trunk. Massive blood flow to the lungs causes pulmonary hypertension. Early cyanosis and CHF.

Front 664

VSD

Back 664

Communication between ventricles. Large defect leads to pulmonary hypertension and Eisenmenger syndrome. Systolic murmur.

Front 665

ASD

Back 665

Communication between atriums. Associated with fetal alcohol syndrome.

Front 666

PDA

Back 666

Communication between aorta and pulmonary artery. Associated with congenital rubella. During pregnancy PDA is kept by PGE2. Close with indomethacin. Machinery murmur. Eisenmenger syndrome.

Front 667

Dilated cardiomyopathy

Back 667

Idiopathic, postpartum, alcohol, Coxackie B infections, doxorubicin and cocaine. Presents as CHF with decreased ejection fraction

Front 668

Hypertrophic cardiomyopathy

Back 668

Cause of death in young athletes. Autosomal dominant. Asymetrical hypertrophy in ventricular septum. Decreased compliance and stroke volume. Rx.: increase preload with beta blockers (decrease HR)

Front 669

Carcinoid heart disease

Back 669

Right sided endocardial and valvular fibrosis secondary to serotonin in patients with carcinoid metastasis to liver. Skin flushing, diarrhea, cramping, bronchospasm, wheezing, telangiectasia

Front 670

Fibronectin

Back 670

Binds collagen, fibrin and integrins; adhesion glycoprotein of extracellular matrix; chemotactic for fibroblasts and endothelial cells

Front 671

VEGF

Back 671

vascular endothelial growth factor; important in angiogenesis

Front 672

FGF

Back 672

Fibroblast growth factor; important in angiogenesis

Front 673

PDGF

Back 673

Stimulates granulation tissue formation; stimulates proliferation of smooth muscle, fibroblasts and endothelium

Front 674

Laminin

Back 674

Adhesion protein in basement membranes; binds type IV collagen, integrins and ECM components