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536 Cards in this Set

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- like endometriosis, but the endometrium grows in the myometrium
- foci of endometrium deep in the myometrium
acute endometritis
acute inflammation of the endometrium
chronic endometritis
- chronic inflammation of the endometrium
metastatic endometrial cancer
- see abnormal glands and cytologic atypia
crohn's disease
- chornic inflammatory condition that most commonly affects the terminal ileum
- sharp demarcation of involved segments of bowel (cobblestone)
- non-caseating granulomas
- final segment of the small intestine
- most often involved in Crohn's disease
- think ileocecal valve
adenocarcinoma of the colon
- develops most commonly after 60 years
- fungating, ulcerated mass protruding into the lumen
- ring-like infiltrating lesion that narros the intestinal lumen
what aggravates varicose veins?
- ncreased venous pressure in legs
- associated with puberty, pregnancy, and onset of menopause in women
- if you stand perfectly still (like at a cash register- the muscles sqeezing blood out of your legs do not work)
why does pregnancy cause varicose veins?
- the fetus compresses large veins of the abdomen
how does CSF return to the venous system?
- drains through the arachnoid granulations into the superior sagittal dural venous sinus
what is an obstructive or noncommunicating hydrocephalus?
- obstruction limits the flow of CSF
what is a nonobstructive or communicating hydrocephalus?
- caused by oversecretion of SCF (e.g. choroid plexus papilloma)
- or by CSF absorption problems in the subarachnoid space or even at the arachnoid granulations (eg. tumor in the subarachnoid space and menigitis that obliterates the arachnoid granulations)
what are fibrillins?
- large, EXTRACELLULAR glycoproteins assocaited with both elastic and non-elastic microfibrils
- fibrillin is the major component of microfibrils found in the extracellular matrix
describe the elastic fiber that makes up tissues such as blood vessels, skin, uterus, and lung
- elastic fibers consist of a central core made of elastin, surrounded by a peripheral network of microfibrils
- peripheral microfibrillar network that surrounds the core is made mainly of fibrillin, a secreted glycoprotein that associates with either itself or with other components of the ECM
mid-systolic click
mitral valve prolapse
what do autoantibodies to adhesion proteins cause?
- autoantibodies to desmosomal or hemidesmosomal proteins can lead to pemphigus vulgaris or bullous pemphigoid
- autoimmune blistering disorder
- 4 types:
1. pemphigus vulgaris (80%)
2. pemphigus vegetans
3. pemphigus foliaceus
4. pemphigus erythematosus
pemphigus vulgaris
- most common of the pemphigus diseases
- involves mucosa and skin, esp of the scalp, face, axilla, groin, trunk and points of pressure
- may presenta s oral ulcers
- primary lesions: superficial veiscles and bullae that rupture easily
- autoantibody to desmoglein 3: component of desmosomes that bind keratinocytes together
- disoolution or lysis of the intercellular adhesion cites within a squamous epithelial surface
- seen in pemphigus vulgaris
bullous pemphigoid
- relatively common autoimmune vesiculobullous disease
- lesions are filled with clear fluid on normal or erythematous skin
- found on inner thighs, felxor surfaces of forearms, axillae, groin, and lower abdomen
- caused by antibodies direceted at proteins at the dermal-epidermal juncgion : linear deposition of Ig
how do you differentiate bullous pemphigoid from SLE?
- linear zone of deposition of Ig in bullous pemphigoid
- granular deposition of Ig in SLE
name the bacteria involved in subacute bacterial endocarditis
Hemophilus aphrophilus
Actinobacillus actinomycetemcomitans
Cardiobacterium homnis
Elkenella corrodens
Kingella kingae
Cardiobacterium hominis
- commonly resistant to erythromycine
- may also be resistant to penicillins
- part of the HACEK group for subacute endocarditis
- gram neg
actinomyces israelii
- gram- positive
- anaerobic rod
- found in gingival crevices
- cause of actinomycotic mycetomas (lumpy jaw): draining abscesses along jaw line caused by poor oral hygiene or trauma
Coxiella bumetii
cause of Q fever, which may cause chronic subacute endocarditis
- intracellular pathogen associated wtih livestock breeding operations
streptococcus mutans
- normal oral flora
- can enter blood stream and cause subacute endocarditis
- gram positive
how do granulomas form?
- interactions between T cells and macrophages
- activated macrophages acquire an epithelial like appearance (epithelioid macrophages) and can fuse to form multinucleated giant cells
- process begins by uptake of foreign antignes by macros -> present to T_helper cells -> recruit more T cells by IL-2 secretion -> activate macros via INF-g -> INF-g causes macros to become epithelioid and multinucleated cells.
- epithelioid macros secrete IL1 and TNF-a to increase leukocyte recruitment and to mediate the acute-phase response
why do macrophages secrete NO?
- microbial killing
- but this can also cause tissue damage from NO free radicals
mixed connective tissue disease
- see Anti-RNP (ribonuclear protein) antibodies; low titer RF and anti-ssDNA
- symptoms suggestive of SLE, plymyositis, RA, and systemic sclerosis
- no renal disease; highly responsive to corticosteroids
- 85% of patients have lung involvment
anti-centromere antibody
- marker for the CREST variant of scleroderma (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia)
marker for scleroderma or systemic sclerosis (multisystem disorder with fibrosis of skin, blood vessels, and viscera
- anti-Scl70: directed against DNA topoisomerase I
anti-histone antibody
- (without other autoantibodies)
- marker for drug-induced lupus
- associated with hydralazine and procainamide
- treats high BP
- inhibits membrane-bound enzymes that form reactive oxygen species, such as superoxides. Excessive superoxide counteracts NO-induced vasodilation.
- commonly used in preeclampsia
- can cause drug induced lupus
large furrowed tongue
- acromegaly
- other features: deep, husky voice, excessive perspiration, jaw protrusion, large hands and feet with joint symptoms, peripheral neuropathies, headaches secondary to underlying pituitary tumor, coarse facial features
what are clinical signs of Addison's disease?
- tanning on non-sunexposed skin
- black freckles on face, neck, and shoulders
- areas of vitiligo
physical exam findings for Cushing's syndrome
- buffalo hump
- purple striae on abdomen
physical exam findings for hyperthyroidism
- ptosis
* pretibial myxedema
a condition characterized by swelling of the skin and other tissues, particularly around the eyes and cheeks, caused by extreme deficiency of thyroid hormone
findings on physical exam for hypothyroidism
- facial puffiness
- dry skin
- brittle hair
how do you diagnose pituitary GH excess?
- documentation of elevated serum GH and IFG-1 levels
- failure to suppress GH production after an oral load of glucose -> great test for acromegaly
what are the common LFTs?
# 1 Standard liver panel
* 1.1 Albumin (Alb)
* 1.2 Alanine transaminase (ALT)
* 1.3 Aspartate transaminase (AST)
* 1.4 Alkaline phosphatase (ALP)
* 1.5 Total bilirubin (TBIL)
* 1.6 Gamma glutamyl transpeptidase (GGT)
ALT (alanine transaminases)
aka SGPT (serum glutamic pyruvic transaminase) or alanine aminotransferase (ALAT)
- an enzyme present in hepatocytes When a cell is damaged, it leaks this enzyme into the blood, where it is measured. ALT rises dramatically in acute liver damage, such as viral hepatitis or acetaminophen overdose.
- Elevations are often measured in multiples of the upper limit of normal (ULN).
- The reference range is 0- 50 U/L
AST (aspartate transaminases)
aka Serum Glutamic Oxaloacetic Transaminase (SGOT) or aspartate aminotransferase (ASAT)
- is similar to ALT in that it is another enzyme associated with liver parenchymal cells. It is raised in acute liver damage, but is also present in red cells, and cardiac and skeletal muscle and is therefore not specific to the liver. The ratio of AST to ALT is sometimes useful in differentiating between causes of liver damage: The reference range is 0-45 U/L in most laboratories.
ALP (alkaline phosphatase)
an enzyme in the cells lining the biliary ducts of the liver. ALP levels in plasma will rise with large bile duct obstruction, intrahepatic cholestasis or infiltrative diseases of the liver. ALP is also present in bone and placental tissue, so it is higher in growing children (as their bones are being remodelled). The reference range is usually 40-150 U/L.
how do you differentiate staph from strep?
- strep line up on gram stain
- staph appears in clusters (staff of a hospital)
- staff is catalase positive
what does catalase do?
- converts H2O2 to hydrogen peroxide, which is used by macrophages and neutrophils) into H2O and O2
- to test for catalase, a wire loop si rubbed across a colony of gram-pos cocci and mixed with H2O2. if you see bubbles, than it's catalase positive
what are the important strep pathogens?
- lancefield group A, B, and D
- streptococcus pneumoniae
- Strettococcus viridans (group)
Group A beta-hemolytic strep (aka streptococcus pyogenes)
- have lancefield group A antigen
- beta hemolytic (completely lyse RBCs)
- pyogenes = pus producing
- cause strep throat, scarlet fever, rheumatic fever, and post-stretococcal glomerulonephritis
- C carbohydrate, M protein, streptolycsin O, S, pyrogenic exotoxin, streptokinase
which components of the group A beta-hemolytic strep (strep pyogenes) are antigenic?
- C carbohydrate: has lancefield group A type C carbohydrate
- M protein (80 types): major virulence factor. inhibits the activation of complement and protects the organism from phagocytosis
- plasma cells can generate antibodies against the M protein: bind to M protein (oposonization) -> destruction by macrophages and neutrophils
which enzymes seen in group A beta-hemolytic strep (strep pyogenes) contribute to its pathogenicity?
- streptolysin O: (O for oxygen labile: inactivated by O2). destroys RBCs (B-hemolytic). antigenic: after infection, anti-streptolysin O (ASO) antibodies develop
- Streptolysin S: S for O2 stable. responsible for hemolysis but not antigenic
- Pyrogenic exotoxin (aka erythrogenic toxin): can cause scarlet fever and TSS
- streptokinase: activates plasmin and breaks up fibrin clots, hyaluronidase, DNAases, anti-C5a peptidase
which diseases are caused by local invasion and/or exotoxin release by group A b-hemolytic strep (strep pyogenes)?
1. pharyngitis
2. skin infections
3. scarlet fever
4. TSS

can also cause 2 delyaed antibody mediated diseases:
1. Rheumatic fever (caused by self reactivity due to molecular mimicry. T cells target heart valves
2. Glomerulonephritis
describe skin infections caused by group A beta-hemolytic strep (strep pyogenes)
- folliculitis, cellullitis, impetigo (vesicular, blistered eruption -> crusty and flaky and found around mouth)
- treated with dicloxacillin, which covers both group A strep and stap aureus (both which cause skin infections)
- necrotizing fasciitis
necrotizing fasciitis caused by group A strep
- some strains have M proteins that block phagocytosis -> strep can go through a break in the skin, and follow a path along fascial planes
- treat with penicillin G and clindamycin (shuts down strep metabolism and blocks toxin production)
- covers both group A beta hemolytic strep and staph aureus
- penicillinase resistant penicillin
- used to treat skin infections
- used against:
- aerobic gram +: staph, strep
- anaerobic gram -: bacteroides
what can cause nectrotizing fasciitis?
- group A b-hemolytic strep
- staph
- clostridium species
- gram-neg enterics
scarlet fever caused by group A b-hemolytic strep
- caused by pyrogenic toxin (aka erythrogenic toxin)
- rash begins on trunk and neck, and then spreads to extremities, sparing the face
toxic shock syndrome
- caused by group A beta-hemolytic strep (release of pyrogenic/erythrogenic toxin). treat with penicillin G and clindamycin (that shuts down strep metabolism and toxin production)
- caused by staph aureus
when does acute post-streptococcal glomerulonephritis occur?
- one week after infection of pharynx or skin by nephritogenic streains o beta-hemolytic group A strep
which bugs are associated with neonatal meningitis?
- E .coli
- Listeria monocytogenes
- Group B strep (Strep agalactiae)
which gram positive bugs cause endocarditis (acute and subacute)
- staph aureus: acute
- strep viridans: subacute
- group D strep: subacute
how do the streptococci work together to establish SBE?
- strep pyogeneis causes rheumatic fever, which damages heart valves
- now, viridans strep or group D strep can more easily adhere to the valves and cause SBE
streptococcus pneumoniae (aka the penumococcus)
- no lancefield antigen
- bacterial pneumo and meningitis in adults
- otitis media in children
- DIPLOCOCCI under microscope (lancet shaped)
- major virulence factor: polysaccharide capsule that protects from phagocytosis. however, there are 84 different capsule serotypes
how do you test for pneumococcus?
1. Quellung reaction: pneumococci are mixed with antiserum (antigens to capsular antigens) and methylene blue, the capsule will swell
2. Optochin sensitivity: to tell strep pneumo from strep viridans (both a-hemolytic and green). strep pneumo is inihibited by optochin disk
- has 25 of the most common capusular polysaccharid antigens in strep pneumo
- given to immunocompromised or elderly people
how do you treat strep pneumo?
- starting to become resistant to penicillin
- however, can still treat with high dose penicillin
- cephalosporins are effective against those with intermediate level resistance
what is unique about s. pyogenes (group A betahemolytic strep) in trying to ID it?
- only B-hemolytic strep that is bacitracin sensitive
what is the Quellung reaction used for?
- testing for encapsulated bacteria:
- Strep penumo
- H. influenzae
how does strep pneumo attach to respiratory mucosa?
- it has teichoic acids in its envelope and produces IgA protease
- IgA protease cleaves the Ig molecules, leaving the Fc parts to coat the bacterium -> allows strep pneumo to bind to Fc receptors on mucosal cells!
- alpha-hemolytic exotoxin produced by strep pneumo
- mediates damage to respiratory epithelium during infection
which group of streptococci has an M protein?
- Group A
- used to type the bacterium
- helps inhibit phagocytosis
what are the AIDS defining illnesses?
- mycobacterium avium complex infection
- mycobacterium tuburculosis
- mycobacterium kansasii
- recurrent salmonella septicemia
which bacteria are acid fast?
- mycobacteria
- nocardia
- caused by high lipid content in cell walls
what is invovled in mycobaterial virulence?
- mycosides: one calss of lipid that only acid fast organisms have
- myocosides is a mycolic acid (a large fatty acid) bound to a carbohydrate, forming a glycolipid
- cord factor is a mycoside found in M. tb (causes parallel growth of bacteria -> look like cords
bacillary angiomatosis
- caused by bartonella henselae
- associated with early symptomatic HIV period, but does not diagnose full-blown AIDS
hairy leukoplakia
associated with early symptomatic period of HIV infection, but not the diagnosis of full-blown AIDS
rare but lethal food-borne infection that has a devastating mortality rate of 25%
- caused by listeria monocytogenes
- associated with early symptomatic period of HIV infection, but not in diagnosis of full-blown AIDS
thrush (aka oral candidiasis)
- associated with early symptomatic period of HIV infection, but not in the diagnosis of full-blown AIDS
what does candida albicans (candidiasis) cause in normal hosts?
1. oral thrush: patches of white exudate with reddish base over the mucous membranes of the mouth. hard to scrape off
2. vaginitis: yeast infection
3. diaper rash
what does candida albicans (candidiasis) cause in the immunocompromised patient?
1. esophagitis: burning substernal pain worse with swallowing
2. disseminated: can invade bloodstream and organs. may be able to see white fluffy candidal patches in the retina.
- diagnosis made with KOH preparation of skin scrapings or with stains.
heberden's nodes
- bony swellings in osteoarthritis. thought to be related to repetitive joint trauma
- seen in women.
- prominent osteophytes at the distal interphalangeal joints
secondary osteoarthritis
- appears in younger people with predisposing condition: micortrama to joints, congenital joint deformity, underlying systemic disease like diabetes, ochronosis, hemochromatosis, or obesity
- invovles predisposed joints (shoulder or elbown in baseball players)
- targets articular cartilage
- in most patients, insidious onset with aging
- invovlement seen in proximal and distal IP joints, sometimes without inflammation, hips, knees, lower lumbar and cervical vertebrae
septal arthritis (causitive agents)
- acute suppurative arhritis
- caused by: gonococcus, staph, strep, H. inf, gram - (E. coli, salmonella, pseudomonas)
- H. inf: <2 yo
- S. aureus: older children and adults
- gonococcus: late adolescence and young adults
clinical presentation of suppurative arthritis
- suddent development of acutely painful, hot, swollen joint with restricted range of motion
- in 90% of nongonococcal cases, infection invovles only single joint: esp knee (also, hip, shoulder, elbow, wrist, and sernoclavicular joints)
which viruses can cause transient arthralgias and arthritis?
- parvovirus B19
- rubella
- hep C
what is the smallest species of RNA?
- tRNA
- larges: mRNA and heterogeneous nuclear RNA (hnRNA)
- rRNA is synthesized from a 45S precursor, which is cleaved inot 5.8 and 28S rRNA
Respiratory syncytial virus (RSV)
- part of the paramyxoviridae family
- cuases respiratory infections and contains the F-protein that causes formation of multinucleated giant cells (syncytial cells)
- does not have HA and NA glycoproteins
- primary cause of pneumo in young kids
- treated with palivizumab
- monoclonal antibody against RSV directed to the fusion protein of RSV, thereby preventing infection of the host cell
- selective costimulation modulator that inhibits T cell activation
- treats RA
- monoclonal antibody with high affinity for the IL-2 receptor on activated T cells
- used to prevent tissue rejection in transplant patients
- induces lipocortin
- inhibits phospholipase activity and prostaglandin synthesis
- can inhibit transcription of proinflammatory cytokines
- inhibuts T cell proliferation by binding to serine-threonine kinase (mTOR) which is necessary for cell cycle progression
Addison disease
- severe adrenal disease: primary deficiency of aldosterone and cortisol due to adrenal atrophy .
- causes hypotension, skin hyperpigmentation (MSH, a byproduct of incrased ACTH production from POMC)
- caused by idiopathic adrenal atrophy (autoimmune lyphocytic adrenalitis), TB, metastatic tumor, infection
what serum abnormalities do you see with Addison's disease?
- decreased Na, Cl, glucose, bicarb
- increased K
what do patients with Addison's disease report?
- fatigue, GI distress
- maybe low sugars from impaired glucocorticoid levels
what can gastric adenocarcinoma cause regarding the ovaries?
- metastatic invasion of the ovaries -> failure of menstruation
- parasitic infection caused by larvale cysts of tapeworm Taenia solium (the 'neuro' part comes with invasion into the CNS
- seen in people from mexico, south central america, philippines, and southeast asia
- on radiology, cysts act as space-occupying lesions that can cause hydrocephalus and/or seizures
- caused by entamoeba histolytica
- present with diarrhea (bloody), RLQ abdomainal pain, and fever
- abscesses in liver are a complication due to invasion of the portal venous sysstem by the amoeba
- produces neonatal infections and infections in immunocompromised patients (CD4 < 100)
- most common presenation: chorioretinitis producing floaters, visual field deficiets, and painless loss of vision
- can also produce encephalitis and calcifying lesions in the CNS
- parasitic cestode infection caused by Echinococcus granulosis or E. multilocularis
- hydatid cyst disease
- patient ingests eggs from dogs and becomes intermediate host
- cysts are classically in the liver, are calcified.
- patient shows eosinophilia
- protozoan infection from eating undercooked meat or exposure to cat feces
- primary toxo is asymptomatic (or sometimes mono like symptoms)
- in patients with AIDS, can cause ring-enhanced focal brain lesions and pneumonia
which 5 intestinal protozoa cause diarrhea?
- entamoeba histolytica (ameoba): bloody
- Cyclospora cayetanensis: non-bloody
- Cryptosporidium and Isospora belli (severe diarrhea in immunocompromised patients)
what does metronidazole kill?
- entamoeba histolytica
- giardia lamblia
- trichomanas vaginalis
- anaerobic cocci and bacilli: bacteroides fragilis, C. difficile, and gardnerella vaginalis.
- aka Flagyl
what are some side effects of metronidazole (flagyl)?
- causes disulfiram (antabuse) like effects: upset stomach after drinking
- can end up with metallic taste in mouth
what is the most common CNS infection in AIDS patients?
- toxoplasma encephalitis
- organisms that cross the blood-placenta barrier
TO (toxoplasmosis)
R (rubella)
HE (Herpes, HIV)
S Syphilis
how do you treat acute toxoplasmosis?
- sulfadiazine plus pyrimethamine
- can cause chorioretinitis and blindess
- can also cause esophagitis (pain with swallowing) and diarrhea)
what do you watch out for in visual compromise in AIDS patients?
- retinal lesions of CMV
- brain masses of toxoplasmosis and lymphoma
- infected cells become swollen (cytomegaly)
- part of the herpesviridae family
- 80% asymptomatic
- part of TORCHES. most common viral cause of mental retardation
- can cause CMV mononucleosis (like mono from EBV)
- can reactive in the immunocompromised patient
what are general characteristics of teh herpesviridae group?
1. can develop a latent stage
2, members in the sub-family alpha have a cytopathic effect on cells -> become multinucleated giant syncytial cells with intranuclear inclusion bodies
3. are held at bay by cell-mediated immune response
which are the alpha sub group herpesviridae? which are the beta subgroup? gamma subgroup?
- herpes simplex 1 and 2
- varicella zoster
- have most cytopathic effect: separation of epithelium and causes blisters. multinucleated giant cells and intranuclear inclusion bodies
beta: CMV
gamma: EBV
how does CMV infection present in an AIDS patient? how does CMV infection present in a bone marrow transplant patient?
- CMV retinitis
- CMV penumonitis
how does EBV cause Burkitt's lymphoma?
- EBV infects B cells and binds to C3d receptor -> internalized -> EBV changes the infected cell so that the cell does not follow normal growth controls (aka TRANSFORMED CELL) -> pass on copies of EBV DNA to cell progeny
what is Burkitt's lymphoma?
- a B cell lymphoma affecting children in central africa
describe heterophile antibody seen in mononucleosis caused by EBV
- antibody against EBV that cross reacts with and agglutinates sheep RBCs
- can be used as a rapid screening test (Monospot test)
- part of the herpesviridae family
- appears to cause Kaposi's sarcoma
treatment and prevention of HSV-1
- acyclovir
- valacyclovir
- famciclovir
- friluridine eye drops for corneal infection
treatment and prevention of HSV-2
- acyclovir
- valacyclovir
- famciclovir
- condoms
treatment and prevention of VZV
- acyclovir
- valacyclovir
- famciclovir
- chickenpox vaccine
- zoster Ig
treatment and prevention of CMV
- gancyclovir
- foscarnet
- cidofovir
- fomiversen
treatment and prevention of EBV
- supportive
define pleiotropy
single gene influences multiple phenotypic traits. Consequently, a new mutation in the gene will have an effect on all traits simultaneously.
Retinoblastoma and pleiotropy
- pleiotropy is seen in Rb: mutation carriers can also develop other cancers (e.g. osteosarcoma)
A highly penetrant gene will express itself almost regardless of the effects of environment, whereas a gene with low penetrance will only sometimes produce the symptom or trait with which it has been associated.
how are most diabetic patients started on oral hypoglycemics?
- usually started on a sulfonylurea or metformin
- sulfonylureas: an insulin-secretagogue. contraindicated with those with hepatic and liver dysfunction
- glipizide primarily undergoes hepatic clearance and should be used in patients with renal dysfunction
- lispro (short acting)
- insulin (short acting)
- NPH (intermediate)
- Lente (long acting)
- ultralente (long acting)
- binds insulin receptor (TK activity)
- liver: increase glucose stored as glycogen
- muscle: increase glycogen and protein synthesis, K uptake
Fat: aids TG storage
- AEs: hypoglycemia, hypersensitivity rxn (rare)
1st gen:
- Tolbutamide
- Chlorpropamide
2nd gen:
- Glyburide
- Glimepiride
- Glipizide
- close K channel in B-cell membrane -> depolarization -> Ca influx -> triggers insulin release
- AEs: 1st gen: disulfiram like effects; 2nd gen: hypoglycemia; contraindicated in those with hepatic dysfunction
- metformin
- mech unknown
- decrease gluconeogenesis
- increase glycolysis
- decrease serum glucose
* can be used in patients wtihout islet function
- AE: lactic acidosis (esp in pts with heart, liver, hypoxia or acidotic disorders). don't want to use if creatinine is even mildly elevated
- pioglitazone
- rosiglitazone
- increase insulin effects on target cells (bind to PPAR-g receptors)
- AEs: weight gain, edema, hepatotoxicity (troglitazone no longer used)
a-glucosidase inhibitors:
- acarbose
- miglitol
- inhibit intestinal bursh border a-glucosidases
- AEs: GI disturbances (like lactose intolerance)
- ectoparasite infection from the mite sarcoptes scabei
- female mite burrows into the stratum corneum to lay eggs
- itchiness from delayed hypersensitivity reaction to mite, feces, or eggs
- linear, streaks (0.2-0.6cm) on interdigital skin, palms, wrists, periareolar skin of women, and genital skin of men
how do you treat scabes?
- topical scabicides like permethrin cream
cutaneous larva migrans
- caused by larva of dog or cat hookworm Ancylostoma brazillense
- causes longer burrows than scabes that involves feet, legs, buttocks, or back
molluscum contagiosum
- due to viral infection
- causes papules, but not tracts
- lice.
- live on top of the skin. do not burrow in
- causes erythema, papules, and pustules of the central face
Arachnida (spiders, scorpions, ticks, and mites)
Insecta (lice, bedbugs, bees, wasps, fleas, flies, and mosquitos)
Chilopoda (centipedes)
how do you treat Hyperaldosteronism?
- spironolactone: K+ sparing diuretic that acts as an aldosterone antagonist
why do you have decreased hematocrit with Conn's sydnrome?
- water retention dilutes blood
where do you must often get secretion of ectopic ACTH?
- small cell carcinoma of the lung
- can cause Cushing's syndrome
what causes release of ACTH?
- corticotropin-releaseing hormone (CRH)
what can cause secondary diabetes?
- cushing's disease
clinical manifestations of Cushing's syndrome
- buffalo hump
- striae
- thin, fragile skin
- osteoporosis from bone resorption
- infections
- CNS problems: mood swings
- hirsutism and menstrual anormalities
how do you differentiate pituitary Cushing's syndrome from ectopic Cushing's syndrome?
- for pit CS, dexamethasone suppression at high levels will cause reduction of ACTH
- for ectopic CS, even high levels will not alter ACTH
- adrenal tumor CS has low ACTH to begin with, and will not be affected by either high or low dose dexamethasone
why do you have higher blood pressure in Cushing's syndrome?
- crossover mineralocorticoid activity of the increased plasma cortisol
- cortisol also makes blood vessels more responsive to catecholamines, which could increase TPR
21-hydroxylase deficiency
- accounts for >90% of the cases of congenital adrenal hyperplasia (CAH)
- defective conversion of progesteronn to 11-deoxycorticosterone
- CYP21B mutation
1. salt-wasting (no aldosterone)
2. virilization (high ACTH cause increased testosterone production)
3. non-classic adrenogenitalism: androgen excess during childhood and puberty (otherwise nl)
what should you suspect in any neonate with ambiguous genitalia?
- CAH (congenital adrenal hyperplasia) caused by high positive feedback by ACTH from decreased cortisol
which exocrine pancreatic defects can cause secondary diabetes?
- chronic pancreatitis
- pancreatectomy
- neoplasia
- CF
- hemachoromatosis
which endocrinopathies can cause diabetes?
- acromegaly
- Cushing syndrome
- hyperthyroidism
- pheochromocytoma
- glucagonoma
which infections can cause diabetes?
- Coxsackie virus B
- cytomegalovirus
which drugs can cause diabetes?
- glucocorticoids
- TH
- a-interferon
- protease inhibitors
- b-adrenergic agonists
- thiazides
- nicotinic acid
- phenytoin
which genetic syndromes are associated with diabetes?
- Down syndrome
- Kleinfelter syndrome
- Turner sydnrome
what causes secretion of renin?
- b-receptor activity on juxtaglomerular cells
prion disease
- rapidly progressing ataxia and dementia
- spongiform encephalopathy with axonal degeneration, neuronal loss, gliosis, and astrocytic proliferation from accumulation of Proteinase K resistant PrP^sc
lewy bodies
- seen in PD and dementia with Lewy bodies
- slow progression
- dementia with Lewy bodies usually invovle visual hallucinations and delusions
huntington's disease
- autosomal dom, fatal, progressive degeneration and atrophy of the striatum (caudate nucleus and putamen) and frontal cortex with neuronal deplietion and gliosis
- delayed presentation until 30s and 40s
- trinucleotide repeats
define spongiform encephalopathy
- clusters of small cysts in CNS gray matter without inflammatory response
what is the clinical presentation of Huntington's disease?
- chorea: jerky, hyerkinetic, dystonic movements affecting all parts of the body
- starts between 40s-50s
- motor symptoms precede cognitive impairment (forgetflness and thought and affective disorders)
- increased suicide risk
what are the genetics behind huntington's disease?
- trinucleotide CAG repeat in the HD gene
- the HD gene encodes huntingtin
- loss of striatal inhibitory output to the globus pallidus (outer) -> increased inhibitory input to the subthalamic nucleus -> choreoathetosis
what can impair vertical gase?
- progressive supranuclear palsy
- associated with dysarthria, dysphagia, dementia, gait ataxia, and extrapyuramidal symptoms
- slow progression
progressive supranuclear palsy (PSP)
- truncal rigidity with dysequilibrium and bucal dystonia
- pseudobulbar palsy and abnromal speech
- vertical gaze palsy progressing to palsy of all eye movements
what causes CIN?
- 85% of Cervical intraepithelial neoplasia (cervical cancer) is caused by HPV 16/18
what are the three members of the Papoviridae family?
1. human papilloma virus (HPV)
2. polyomavirus: human BK and JC
3. simian vaculating virus
- O for circular ds DNA
- O for round warts
- O for round cervix
what do you see on histology for CIN?
- enlarged, hyperchromatic nuclei and prominent perinuclear halos
describe E6 and E7 in HPV
- E6 inactivates p53 -> Li-Fraumeni syndrome
- E7 inactivates RB -> retinoblastoma, osteosarcoma and some carcinoams of the breast, colon and lung
Li- Fraumeni syndrome
- germ line mutation of the p53 gene
- can be seen from HPV E6 inactivation of p53
which viruses can inactivate RB?
- simian virus 40
- polyomavirus large T antigens
- adenoviruses EIA protein
- HPV E7 protein
- bidn to the hypophosphorylated form of RB (at the same pocket that normally sequesters E2F transcription factors) -> cell-cycle progression
what causes Burkitt lymphoma?
- malignant transformation of B lymphocytes infected by EBV
- results from translocation of the c-myc proto-oncogene on chr 8 to chr 14, next to the Ig gene region
familial adenomatous polyposis
- results from the inactivation of the APC tumor suppressor gene, which normally functions to inhibit B-catenin
- hereditary
- hugely high risk fo cancer
- 100% of patients are fated to develop carcinoma of the colon by 50
malignant melanoma, genetics
- mutation on chr9p21
- encodes p16 (aka cyclin-dependent kinase inhibitor 2: CDNK-2), which is deleted in melanomas
- lack of functional p16 -> unrestricted phosphorylation of RB -> release of E2F -> controlled cell growth
wilms tumor, genetics
- pediatric kidney cancer that invovles mutation of the WT-1 (wilm's tumor-1) locus
- WT-1 is a transcriptional activator of genes involved in renal and gonadal differentiation
who has an increased risk for wilm's tumor?
1. those with WAGR syndrome: wilms tumor, aniridia, genital abnormalities, and mental retardation
2. those with Denys-Drash sydnrome: gonadal dysgenesis, early onset nephroppathy (diffuse mesangial sclerosis)
3. Beckwith-Wiedemann syndrome: organomegaly, macroglossia, hemihypetrophy, omphalocele, and adrenal cytomegaly
ancylostoma duodenal and necator americanus
- hookworms
- cause anemia
enterobius vermicularis
- pinworm. lives in the large intestine
- gravid females migrate at night into the perianal region to lay eggs.
- put cellophane tape on perianal skin for microscope exam
- clincial: pruritis
- treatment: mebendazole
ascaris lumbricoides
- large, intestinal roundworm
- very common in tropics
- can cause GI distress
trichuris triciura
- whipworm
- can cause rectal prolapse
atonic seizures
- aka drop seizures
- sudden loss of postural muscle tone that only lasts a couple of seconds
- no postictural confusion
- brief seizure can cause head drop; longer seizure can cause falling
how do you treat atonic seizures?
- valproic acid
- conazepam
- felbamate
- givabatrin
- lamotrigine
absence seizures
- aka petite mal seizures
- blank stairs and absence in any change of position
- seen in children
- treated wtih ethosuximide, valproic acid, and clonazepam
myoclonic seizures
- sudden, brief, muscle jerks
- treat with valproic acid and clonazepam
tonic seizures
- sudden onset of sustained axial and limb muscle contraction
- seen in children
tonic-clonic seizures
- aka grand mal seizures
- initial phase of tonic contraction, then clonic phase: relaxation and contraction cycles
- significant postictal phase
- treat with carbamazepine, phenytoin, and valproic acid
Riedel thyroiditis
- aka ligneous (rocklike) stroma
- marked fibrous reaction that destroys most or all of the thyroid gland
- affects older women
- can cause stridor, dyspnea, dysphagia, laryngeal nerve paralysis or hypothyroidism
what do you think of when you see a massive, soft, thyroid gland?
- multinodular goiter
what do you think of when you see a painful and tender thyroid?
- subacute granulomatous (de Quervain) thyroiditis
what do you think of when you see a single large thyroid nodule?
- thyroid adenoma or thyroid cancer
what is the most common cause of maternal death in preeclampsia?
- cerebral hemorrhage
- pulmonary complications (ARDS)
define: chronic PTSD
- symptoms lasting more than 3 months
- persistant flashbacks
where is GH secreted?
- GH is secreted by anti pituitary somatotroph cells in response to hypothalamic GHRH
- GHRH receptors are G protein coupled recetors that activate G_s alpha -> AC -> increase in cAMP
- mutation preventing hydrolysis of GTP by G_s alpha will cuase excess receptor-mediated signaling to Pit -> excess GH secretion
how are the growth effects of GH mediated?
- mediated by IGF-a, which is secreted by the liver
what does somatostatin do to GH release?
- inhibits GH release
which would you prefer to give: T3 or T4?
- T4
- however, will give T3 if need better GI absorption or shorter duration of action (e.g pts undergoing short-term suppression of a thyroid nodule or thyroid cancer pts who need to interupt treatment for chemo
childhood disintegrative disorder
- clinically significant loss of previously acquired skills
- does not let them function well even in sheltered workshops
mixed receptive-expressive language disorder
- a communication disorder in which both the receptive and expressive areas of communication may be affected in any degree
- low scores for Information, Vocabulary and Comprehension If the person has difficulty with spatial concepts, he or she may have arithmetic difficulties, have difficulty understanding word problems and instructions, or have difficulties using words.
Rett's syndrome
- severe impairment
- loss of social engagemetn and previously acquired (before 30 mo) skills, and hand wringing movements
- females only
when do you see an increase in airway resistance?
1. asthma
2. bronchospasm
3. emphysema
4. old age
- decreases FEV1/FVC below nl of 80%
what increases FEV1/FVC
- interstitial fibrosis
- increased radail traction of the airways (airways are held open to a greater extent at any given volume -> reduced resistance)
- increase in elastic recoid makes it difficult to breath deeply
what are the four types of myeloproliferative disorders?
1. chronic myeloid leukemia (CML)
2. poycythemia vera
3. myeloid metaplasia with myelofibrosis (MMM)
4. essential thrombocythemia
when would you get ineffective granulopoiesis?
- suppression of myeloid stem cells (aplastic anemia). will also see anemia and thrombocytopenia
- drugs -> suppress granulocytic precurors
- megaloblastic anemias
- myelodysplastic syndromes
- inherited (Kostmann syndrome)
define myeolodysplastic syndromes
- defective precursors are susceptible to death in the marrow
drugs that cause neutropenia
- drugs are responsible for most of the significant neutropenias
- alkalating agents and antimetabolits
- aminopyrine, chloramphenicol, sulfonamides, chlorpormazine, thiouracil, and phenylbutazone
how do you stimulate granulocyte growth after myelosuppressive chemotherapy?
- give G-CSF: stimulates granulocyte production from marrow precursors
kawasaki disease
also known as lymph node syndrome, mucocutaneous node disease, infantile polyarteritis and Kawasaki syndrome, is a poorly understood self-limited vasculitis that affects many organs, including the skin and mucous membranes, lymph nodes, blood vessel walls, and the heart.
what can cause follicular hyperplasia?
- RA
- toxoplasmosis
- early stages of HIV infection
name the three genetic diseases that promote genomic instability
1. Bloom syndrome
2. Fanconi anemia
3. Ataxia telangiectasia
which conditions are associated with an increased risk for childhood leukemia?
- Down's syndrome
- neurofibromatosis type I
which viruses are associated with neoplasm?
1. HTLV-1: adult T cell leukemia/lymphoma
2. EBV: Burkitt lymphoma
3. KSHV/HHV8: Kaposi's sarcoma
describe how patients with multiple myeloma can die of renal failure
- renal insufficiency is second only to infections as a cause of death in MM
- Bence Jones proeinuria: excreted light chains are toxic to renal tubular epithelial cells
myeloid neoplasms
- derive from hematopoietic progenitor cells for erythrocytes, granulocytes, monocytes, and platelets
CML and polycythemia vera have what in common?
- characterized by hypercellular BM producing all three cell lines
- after 10 years or so, the BM may burn out, and marrow space is replaced by fibrosis
- hematopoiesis moves to extramedullary sites (spleen and liver): myeloid metaplasia with myelofibrosis
which diseases can progress to MMM?
- polycythemia vera
- Note: polycythemia vera does not progress to CML
what do you associated Hairy cell leukemia with?
- massive splenomegaly
- derived from B lymphocytes, not a myeloid stem cell
myelodysplastic syndrome (MDS)
- myeloid disorders featuring maturation defects and ineffective hematopoiesis
- produces cytopenia and acute leukemias
- recall: myeloproliferative disorders produce polycythemia and chronic leukemia
PTH and renal failure
- hyperparathyroidism is a common sequela of chronic renal failure
- high serum phosphate and low serum Ca -> release of PTH
- stimulation of osteoblasts to become osteoclasts -> release Ca into blood
- can cause fractures: renal osteodystrophy
- can lead to osteomalacia and osteitis fibrosa cystica
what do high glucagon levels cause?
- transitory skin rash, anemia, and DM
describe how cortisol can cause increased gluconeogenesis in hepatocytes
- cortisol is a steroid hormone. can diffuse into the cytoplasm and bind to intracellular protein receptor -> increase protein synthesis
describe the intracelluar signalling for glutamate
- receptor for neurotransmitter glutamage induces Ca channel activation after glutamate binding
what is the total body iron pool?
0.5 g is stored in the liver, 98% in hepatocytes
- in hereditary hemochromatosis, iron stores may exceed 50g
what is the classical triad observed in hemochromatosis?
1. pigment cirrhosis with hepatomegaly
2. skin pigmentation
3. pancreatic damage -> DM
- also can see CHF
- blue-gray skin discoloration related to silver poisoning
Wilson's disease
- autosomal recessive
- copper accumulation in liver, brain, and eye
which infections are patients with no spleen susceptible to?
- salmonella
- encapsulated organisms: Strep pneumo (pneumococcus) and H. inf
- common practice to give the pneumococcal vaccine to asplenic patients
what is one method to treat sickle cell anemia?
- hydroxyurea (chemo agent)
- acts as an anti-inf by inhibiting production of WBCs -> reduce inflammation related red cell stasis and sickling
- increases mean RBC volume -> decrease concentration of HbS
- hydroxyurea can also be oxidized by heme groups to make NO -> vasodilator
beta oxidation of fatty acids
- can produce acetyl-CoA, which may be converted to ketone bodies like acetone and b-hydroxybutarate if the acetyl-CoA is not used in the Citric acid cycle
what does the citric acid cycle produce?
- CO2
what does the mitochondrial oxidative phosphorylation pathway produce?
- H2O
what does the pentose phosphate shunt produce?
- ribose-5-phosphate
HLA association with primary hemochromatosis
- HLA-A3
HLA-B27 associations
- psoriasis
- ankylosing spondylitis
- Reiter's syndrome
HLA-DR2 associations
- SLE (also associated with HLA-DR3)
HLA-DR3 associations
- SLE (also associated with HLA-DR2)
- Sjogren's syndrome
- chronic active hepatitis
- T1DM (also associated with HLA-DR4)
HLA-DR4 associations
- T1DM (also associated with HLA-DR3)
- RA
ankylosing spondyloarthritis (rheumatoid spondylitis; Marie-strumpell disease)
- HLA-B27 association
- chronic inflammation of axial joints (esp. sacroiliac joints)
- activation of T cells
- histo: chronic synovitis with destruction of articular cartilage and bony ankylosis
- low back pain, involvement of hips, knees, and shoulders
- bamboo spine
- inability to synthesize apolipoprotein B
- autosomal recessive mutation in microsomal transfer protein (MTP) gene
- defect in triacylglycerol transfer protein -> can't load apoB with lipid -> no chylomicrons or VLDLs formed -> high triacylglycerides in liver
- can also see lipid membrane defects -> burr cells!
describe the clinical presentation of abetalipoproteinemia
- balance problems and difficulties with night vision
- failure to thrive as an infant, diarrhea, and steatorrhea
- poor muscle coordination, ataxia, decreased proprioceptive or vibratory sensation
- low total cholesterol and vit E levels
abetalipoproteinemia explained again
- extremely low cholesterol and triglycerid levels
- abscence of chylomicrons, VLDL, and LDLs
- clinical manifestations come from defects in absorption and transport of fat-soluble VITAMINS (vit E)
what does vit E deficiency cause?
- hyporeflexia
- distal muscle weakness
- ataxia
- decreased vibratory sensation and proprioception
- night blindness
define acanthocytosis
A rare condition in which the majority of the red blood cells are acanthocytes.
how do you treat abetaproteinemia?
- low-fat, high calorie diet (carb rich) with large supplemental doses of fat-soluble vitamines
defect in 7-alpha hydroxylase (CYP 7A)
- decreased bile acid synthesis -> deficiency in vit E
- as bile acid synthesis is decreased, the increase intrahepatic cholesterol levels will downreg LDL receptors -> increase plasma cholesterol
defect in Hormone sensitive lipase (HSL)
- Hormone sensitive lipase initiates the mobilization of store fat by the hydrolytic release of fatty acids and glycerol from their triglyceride form
- defect will cause a decrease in adipocytes -> increase circulating fatty acids and suppress VLDL secretion
defect in lecithin- cholesterol acyltransferase (LCAT)
- causes elevated triglycerides and sometimes elevated cholesterol
defect in lipoprotein lipase
- inability to cleave triglyceride rich particles like VLDL and chylomicrons -> hypertriglyceridemia
what is the most common cause of maternal death in preeclampsia?
- cerebral hemorrhage and pulmonary complications (e.g respiratory distress syndrome)
list the RNA viruses
- retroviridae
- orthomyxoviridae
- paramyxoviridae
- ARthropod BOrne viruses: togaviridae, flaviviridae, bunyaviridae
- picornaviridae
- cold viruses: rhinovirus, and coronaviridae
- diarrhea: rotavirus and caliciviridae
- rabies: rabdoviridae
what are the ARthropod BOrne viruses?
- arbovirueses: togaviridae, flaviviridae, and bunyaviridae
- cause fever and encephaitis
- Paul BUNYAN wears a TOGA and has a rich FLAVOR
what does the picornaviridae group encompass?
- enteroviruses
- hepatitis A virus
- poliovirus
- coxsackie A, B
- echovirus
which viruses cause the common cold? (RNA)
- rhinovirus: common cold
- coronaviridae: 15% of Common colds (SARS!)
- 'Rhino drinking a Corona beer'
which viruses cause diarrhea? (RNA)
- rotavirus
- caliciviridae (inc. Norwalk virus)
- if the calico cat has diarrhea, ROTATE the kitty litter frequently, or rotate the cat off to NORway
what causes rabies?
- rhabdoviridae
which togaviridae members infect humans?
- part of the arthropod borne virus group (arboviruses)
1. alpha viruses: mosquito born and cause encephalitis. WEE (WESTERN EQUINE ENCEPHALITIS), EEE, VEE (venezuala)
2. rubivirus causes rubella (not an arbovirus!)
- 'german measles': contracted by respiratory secretions -> prodrome of fever and flu, followed by maculopapular rash
- rubella in embryo can cause chromosomal breaks: heart defects: patent ductus, interventricular septal defects, pulm art stenosis; eye: cataracts, chorioretinitis; CNS: mental retardation, microencephaly, deafness
- part of the arboviruses (tick-borne)
- spread by mosquito, infects humans and birds
- causes yellow fever (panama canal project: hepatitis, fever, backach, nausea) and Dengue fever
Dengue fever
- caused by falviviridae
- mosquito borne
- seen in tropics (Peurto rico, virgin islands)
- called break-bone fever b/c of painful backache, muscle and joint pain and headache
- a type of arbovirus
- causes fevera dn encephalitis (California encephalitis and Rift Valley fever)
- RNA virus that causes diarrhea (calico cat..)
- infects young kids
- gastroenteritis is indistinguishable from that of rotavirus, including diarrhea, vomiting and fever
Norwalk virus
- a type of calciviridae
- "if your calico cat has diarrhea, rotate the kitty litter or send her off to Norway"
- occurs in adults
- causes diarrhea
- "rotate the calico cat..."
- member of the reovirus family
- number one cause of acute infectious diarrhea
- only cholera causes worse dehydration
- has s SEGMENTED genome (10-11 segments): can undergo genetic shift
how do you treat rotavirus, calciviridae diarrhea?
- supportive -> IV fluids and electrolytes
which viruses can undergo antigenic shift?
- influenza
- rotavirus
HIV: antigenic drift or shift?
- drift- minor mutations due to an error-prone polymerase. not genetic shift
what causes gas gangrene?
- Clostridium perfringens
- remember, clostridium can cause botulism, tetanus, gas gangrene and pseudomembranous colitis
- spore forming gram pos rod
- deep wounds w/ dead tissue -> anaerobic environment
1. celluitis
2. clostridial myonecrosis
gram positive bacteria
2 cocci: staph and strep
4 rods (bacilli):
- 2 spore formers: bacillus and clostridium
- 2 non-spore formers: corynebacterium and listeria
bacillus and clostridium
- spore forming rods (gram +)
- bacillus: aerobic
- clostridium: 'in the closet' anaerobic
cellultis (C. perfingens)
- necrotic skin is exposed to C. perfringens
- moist, spongy crackling consistency due to pockets of gas following infection
clostridial myonecrosis
- caused by C. perfringens
- toxin produced: A toxin- a lecithinase, phospholipase C that lyses erythrocytes, platelets, leukocytes and endothelial cells
- causes massive hemolysis, vascular permeability, bleeding, hepatotoxicity, bradycardia and hypotension
lecithin is usually used as a synonym for pure phosphatidylcholine
- It is needed by every cell in the body and is a key building block of cell membranes; without it, they would harden. Phosphatidylcholine is an important component of the mucus layer in the large intestine.
collagenase and C. perfringins
- the Kappa toxin
- contributes to the hemolysis and necrotizing activity of the but, but is not an important cause of myonecrosis (gas gangrene)
hemolysin and C. perfringins
- seen in the delta, alpha, theta, and nu toxins of C. perf
deoxyribonuclease and C. perfringins
- seen in the V-toxin
- contributes to hemolysis and necrotizing activity, but is not most important cause of myonecrosis (gas gangrene)
Permease and C. perfringins
- expressed by the epsilon toxin
- produced as a protoxin and activated by trypsin
- increases the permeability of the GI tract
- important cause to the development of necrotizing enteritis (not so much for myonecrosis)
what do radial nerve lesions cause?
- wrist drop
what do ulnar lesions cause?
- clawing of the ring and little fingers and wasting of the hypothenar muscles
- st. benedict's sign
what do medial nerve lesions cause?
- seen in carpal tunnel syndrome
- cause atrophy of the thenar eminence
what is Erb palsy?
- like Klumpke paralysis, a bracial plexus injury that can occur during delivery
- caused by damage to upper part of C5-6
- causes a waiter's tip arm (hand outstretched, palm up, like a waiter hinting for gratuity)
what is Klumpke paralysis? (thoracic outlet syndrome)
- like Erb palsy, a brachial plexus injury seen during rough delivery
- usually seen in a breech delivery
- the lower part of the brachial plexus is stretched (C8-T1)
- paralysis of intrinsic hand muscles: can't oppose thumb and pinkie)
- numbness along inner aspect of hand
- resolve after a few months
when is serum alkaline phosphatase elevatd?
- in metastatic prostate cancer due to the osteoblastic activity in the metastatic foci
- can also be slightly increased in osteoarthritis (reactive bone formation) and in liver disease
serum alkaline phasphatase (ALP)
an enzyme in the cells lining the biliary ducts of the liver. ALP levels in plasma will rise with large bile duct obstruction, intrahepatic cholestasis or infiltrative diseases of the liver. ALP is also present in bone and placental tissue, so it is higher in growing children (as their bones are being remodelled).
how do you acquire viral encephalitis?
- mosquito (arthropod) transmission in the summer
- herpes simplex encephalitis is most common encephalitis in US- direct contact with infected tissues
how do viruses that cause gastroenteritis get transmitted?
- fecal/oral
- most of these are naked capsid viruses -> allow them to survive the acidity in the stomach
how are enveloped viruses transmitted?
- parenteral transmission: direct transfusion or transplantation
- sexual transmission is also common
- enveloped viruses are very fragile in the environment
- used to dissolve cholesterol gallstones
- used to treat mild to moderate ulcerative colitis
- MAOI used to reduce apetite
- PGE1 derivated used to treat gastric ulcers caused by NSAIDs
- DA antagonist used as an antiemetic and to promote GI motility in patients with gastroparesis
- synthetic analog of somatostatin that decreases blood flow to the portal system -> decreases variceal bleeding
how do you diagnose defects in the urea cycle?
- hyperammonemia
- patients with deficiency of ornithine transcarbamylase (OTC) or carbamoyl phosphate synthetase (CPS) will have no abnormal plasma aas, except for increased glutamine, aspartic acid and alanine, secondary to hyperammonemia
- to diff OTC from CPS, you will see increased urinary secretion of orotic acid
what will you see in pts with deficiency in argininosuccinic acid synthase?
increased plasma citrulline
what will you see in pts with deficiency in argininosuccinic acid lyase
argininosuccinic acid increase in plasma
what will you see in pts with deficiency in arginase?
- increased plasma arginine
basal cell carcinoma
- usually occur in sun-exposed areas of head, neck and upper trunk
- slow growing
- rarely metastasize
- increased incidence in those with xeroderma pigmentosum (defect in NER: nucleotide excision repair)
- localized mast cell degranulation and resultant microvascular hyperpermeability -> wheals
- follows exposure to antigens (pollens, foods, drugs, insect venum)
verruca vulgaris
- the common wart
- often seen on hands
- caused by EBV
- regress spontaneously in 6mo- 2yrs
how do you treat cholelithiasis?
- ursodiol: primary bile acid that acts by reducing cholesterol secretion into bile
- good at dissolving cholesterol gallstones
- used alone, may take many months to be fully effective, but can be combined with sonic lithotripsy for full effect
- used in combination with atropine to treat diarrhea
- monoclonal TNF-a used to treat crohn's disease
- synthetic somatostatin used to treat diarrhea in endocrine disorders: carcinoid syndrome, gastrinoma, and glucagonoma
- also used to treat variceal bleeding and acromegaly
- pancreatic lipase
- used to treat steatorrhea caused by inadequate pancreatic lipase secretion
- spasmolytic used for cerebral palsy, MS and stroke
define cerebral palsy
- nonprogressive neurologic motor deficit characterized by spasticity, dystonia, ataxia/athetosis, and paresis attributable to insults during the prenatal and perinatal periods
CMV in immunosuppressed patients
- owl eye inclusion is found in cells throughout body, and seen in sloughed off cells in urinary sediment
- CMV is part of the herpesvirus family : ds DNA with envelope from the host nuclear membrane
ds naked DNA icosahedral virus
- papovavirus and adenovirus
what are the viral infections seen in children in order of decreasing incidence?
1. RSV
2. Parainfluenza
3. Rhinovirus
4. Adenovirus
single stranded DNA, naked, icosahedral virus
- parvovirus
- causes erythema infectiosum or slapped cheek fever- a mild febrile illness of children
- smallest DNA virus
ds RNA, naked, icosahedral
reovirus family, inc rotavirus (diarrheal agent in infants)
- calico cat, rotate kitty litter, send to norway!
ss, neg sense RNA, enveloped helical virus
- paramyxovirus
- rhabdovirus
- filovirus
- orthomyxovirus
- bunyavirus
- arenavirus
ss pos sense RNA, enveloped icosahedral virus
- flaviviruses
- togaviruses
- spread by arthropods (part of the arborviruses)
proclorperazine (compazine)
- phenothiazine that blocks DA receptors in the chemoreceptor trigger zone (vomiting center) in the medulla.
- used as an antiemetic during chemo therapy
- cannabinoid that is taken orally and is used as an antiemetic to control nausea induced by chemotherapy
- 5-HT3 antagonist that is used as an antiemetic to control nausea in chemotherapy
- H1 antagonist to treat motion sickness and true vertigo
- progestational agent used to stimulate appetite
- antimuscarinic agent and antiemetic used for motion sickness
- not useful for nausea caused by chemo
- indicated for mild pain and fever, but does not reduce inflammation
- also safe to use in people with ulcers and CAD
aspirin and indomethacin contraindicated in?
- indomethacin: nonselective COX inhibitor
celecoxib contraindicated in?
- COX-2 inhibitor
- ok to use if you have a PUD
- but contraindicated for coronary disease
- indicated for moderate to severe pain associated with RA rather than for muscle soreness
nl K levels
3.5-5.0 mEq/L
what does severe hypokalemia cause?
- flaccid paralysis
- hyporeflexia
- tetany
- even rhabdomyolysis
bumetanide and ethacrynic acid
- potent loop diurectics
- hypertension and edema for CHF
- AE: hypokalemia
indapamide, metolazone
- thiazide diuretics
- used for initial treatment of hypertension and as adjunctive therapy for treatment of edema associated with CHF
where is one of the earliest and most severely affected cerebral regions in AD?
- hippocampus
- important in short term memory
- lies deep in the hippocampal fissure, medial to the temporal lobe
- projects to the mammillary bodies through the fimbria, which then project to the thalamus
cingulate cortex
- connects the entorhinal cortex (temporal lobe) and hippocampus
describe the hormone levels right before menstruation
- sex steroid levels are low (progesterone, estradiol), but gonadotropin levels (esp FSH) begin to rise slightly
- basal body temp is high during the luteal phase, but falls precipitously before the onset of menstruation
when are increased inhibin levels seen?
- in the middle of the luteal phase
- drop to low levels just before menstruation
when does LH peak?
- 36 hours before ovulation
when does the basal body temp increase?
- after ovulation, due to the metabolic effects of progesterone produced by the corpus leteum
describe compression of the oculomotor nerve (CN III)
- compression first affects the peripheral areas of the nerve, which carry PARASYMPATHETIC info
- central areas have somatomotor info
when do you see upward herniation of the cerebellum?
- seen in patients with posterior fossa lesions
what indicates good prognosis in breast cancer patients?
- tumors that express certain growth factors like estrogen, progesterone, and the HER2-type epidermal frowth factor (EGF) receptors make the lesion more susceptible to:
- estrogen receptor antagonist tamoxifen
- HER2EGFR blocker trastuzumab (Herceptin)
describe EGFR receptors
- are tyrosine kinase receptors seen on the cell surface
duodenal atresia
- seen more often in Downs babies
- failure to recanalize the lumen of the duodenum during formation of the primitive gut -> intestinal obstruction -> bilious vomiting with proximal stomach distension (double-bubble sign)
- antenatal clue: polyhydramnios
define: choledocholithiasis
- presence of stones within the bile ducts of the biliary tree
- not the same as cholelithiasis (stones in the gallbladder)
- can cause obstruction, pancreatitis, cholangitis, hepatic abscess, seciondary biliary cirrhosis and acute calculous cholecystitis
define: cholangitis
- bacterial infection of the bile ducts
biliary atresia
- complete obstructin of the lumen of the extrahepatic biliary tree within the first 3 months of life
- bile duct undergoes luminal occlusion followed by recanalization. if this fails -> extrahepatic or intrahepatic atresia
- see direct (conjugated hyperbilirubinemia) in newborn
what are the two types of biliary atresia?
1. fetal form: associated wtih other anomalies from ineffective establishment of laterality of thoracic and abdominal organ development (malroation of abdominal viscer)
2. perinatal form: virus destroys normal biliary tree
annular pancreas
- ventral and dorsal pancreatic buds abnormally fuse -> compression of duodenum and obstruction
- bilious vomiting
Meckel dierticulum
- persistent remnant of the vitelline duct
- typical presentation: painless brick-red rectal bleeding at 2 years of age
what happens from increasd distention of the small intestine?
- increase in motility through reflex mechanisms -> can cause diarrhea
- blood volume contraction and release of vasoactive substances (bradykinin and VIP) can cause hypotention and reflex tachycardia
Ewing sarcoma
- peak incidence 10-20 yo
- can cause lytic destruction of bone or subperiosteal reactive bone formation
- EWS-FLI1 fusion gene from t(11;22)
- a malignant small round cell tumor (along with PNET)
Giant cell tumor
- seen in 20-40 year olds
- thought ot have monocyte-macrophage lineage
- osteoclast type giant cells have 100 or more nuclei!
- usually arise aroudn the knee
why did children die of CF in the past?
- malnutrition
- now treated with pancreatic enzyme supplements
- gated by ATP hydrolysis and involves regulation by protein kinase A phosphorylation
- has wo cytoplasmic nuceotide bidning domains (NBD) and a regulatory domain (R domain_ that has protein kinase A and C phosphorylation sites
nl pH levels
nl pCO2 levels
33-45 mmHg
nl HCO3- levels
22-28 mEq
describe metabolic acidosis without respiratory compensation
- decreased bicarb
- respiratory system usually compensates by increasing ventilation to decrease pCO2
- if you see an elevated pCO2, respiratory compensation has not yet occured
what organisms can cause honey crusted lesions?
- strep pyogenes
- staph aureus
- you can distinguish the two based on the catalase test
- treat with dicloxacillin, which hits both
what are the three main pathogenic species of Staph?
- staph aureus
- stau epidermidis
- staph saprophyticus
how do you differentiate staph from strep?
- catalase test
- staph have enzyme catalase
how do you tell staph aureus crom other beta-hemolytic cocci?
- elaboration of a golden pigment on sheep culture
which species of staphlococcus is coagulase positive?
- staph aureus
which proteins are found on Staph aureus?
- Protein A (binds Fc portion of IgG). protects from opsonization
- Coagulase
- Hemolysins
- Leukocidins
- Penicillinase
- Novel penicillin binding protein
what is the CAMP test used for?
- used to distinguish group B strep from othe strep
what is the ELEK test?
- immunodiffusion test used to identify toxin-producing strains of Corynebacterium diptheriae
what are the two non-spore forming gram + rods?
- corynebacterium
- listeria
- both infect patients of the pediatric age group
how do you treat listeria monocytogenes?
- ampicillin
- trimethoprim/sulfamethoxazole
when do you use the oxidase test?
- for Neisseria
what is the only pathogenic gram-neg cocci?
- neisseria
how do you diagnose acute pancreatitis?
- elevated serum amylase and/or lipase levels
what causes acute pancreatitis?
- biliary tract disease (usually gallstones)
- alcoholism
hereditary pancreatitis
- recurrent attacks of severe pancreatitis starting in childhood
- cuased by mutation in cationic trypsinogen gene (e.g. PRSS1)
- trypsinogen and trypsin become resistant to inactivation -> autologous digestion and digestion of pro-enzymes
- SPINK1 codes for a pancreatic secretory trypsin inhibitor
- helps prevent autodigestion
- inherited mutations can cause acute pancreatitis
how does constituatively active trypsin seen in acute pancreatitis affect the clotting cascade?
- trypsin converts prekallikrein to activiated form -> activates Hageman factor, the clotting and complement systems
why do you ether to identify virus type?
- ether is a lipid solvent and will inactivate enveloped viruses (has no effect on non-enveloped viruses)
name the single stranded + RNA viruses
Naked: Pciorna, calici
Enveloped: Toa, falvi, corona, retro
what does polio virus infect?
- peyer's patches
- motor neurons
- fecal-oral transmission (but can also be transmitted by respiratory secretions when the virus has colonized the tonsils)
- can cause paralytic poliomyelitis
what are the three disease manifestations of polio?
1. mild illness
2. aspetic meningitis
3. paralytic poliomyelitis
- destroys presynaptic motor neurons of the ant horn and the postsynaptic neurons leaving the horn
describe the polio vaccines
- inactivated vaccine (salk)- formalin killed; given subcutaneously
- oral polio vaccine: attenuated poliovirus. sheds in the feces
- major cause of diarrhea in kids under 2
- part of the reovirus family
- DS, naked
phenylketonuria (PKU)
- classic PKU: homozygous autorecessive
- deficiency of PHENYLALANINE HYDROXYLASE -> causes hyperphenylalaninemia
- by 6 months, will be severely mentall retarded
- can also present with seizures, decreased pigmentation
- can be circumvented by restricting phenylalanine intake
- many stop this diet, and have markedly elevated levels during child-bearing years -> maternal PKU. Majority of kids are mentally retarded, or have congenital heart disease
when is the critical period in development when teratogenic materials affect growing organs the most?
- 3-8 wks of gestation
what causes brain abscesses in children?
- 60% of brain abscesses are caused by middle ear infection
- cerebellar abscesses are associated with middle ear infection
most common agents that cause otitis media
- strep penumo
- h .inf
- moraxella catarrhalis
- chronic OM:
- Pseudomonas aeruginosa, staph aureus or fungus
define empyema
pus accumulation in the lung(s) or other body cavity
what is a complication of bacterial meningitis?
- subdural empyema
what can cause frontal and temporal lobe abscesses?
- middle ear infection and sinusitis
- most common cause of dwarfism
- point mut of FGFR3: defect in cell signalling
- normally, FGF3 inhibits cartilage proliferation
- in achondroplasia, FGFR3 is constantly activated -> suppression of growth
- autosomal dominant
- 80% of cases result from denovo mutations though
- deficiency of lysosomal hydrolases -> lysosomal accumulation of mucopolysaccharids -> will cause short stature adn have chest wall abnormalities and malformed bones
what MS diseases can be caused by mustations in transcription factors?
- eg. homeobox genes
- can cause supernumerary ribs and digits
what do type I collagen mutations cause?
- osteogenesis imperfecta
- deficiency in synthesis of type 1 collagen
- manifests in early infancy or in utero
- NOTE: type 2, 10, and 11 collagen disease affect type 2, 10 and 11 collagen -> components of hyaline cartilage
- very small
- lack of rigid cell wall: cannot be classified as either rod or cocci
- cannot be treated by penicillins or cephalosporins that attack the cell wall
what is the number one cause of bacterial bronchitis and pneumonia in teens and young adults?
- mycoplasma pneumoniae
serodiagnosis of mycoplasma pneumoniae
- atypical pneumo
- pts produce 1 -2 heterophile antibodies: one agglutinates human O+ RBCs @ 4 degrees (cold hemagglutinins), while the other cuases agglutination of the strain Strep salivarius termed strain MG (the strep MG agglutinins)
- susceptible to the macrolide family of antibiotics (erythromycin, clarithromycin)
if you have bowel perforation, which antibiotics would you administer?
- clindamycin for the aerobic gram +
- aminoglycosides for the aerobic gram -
what causes pseudomembranous colitis?
- clindamycin and some penicillins
- natural flora destroyed -> C. difficile will secrete exotoxin -> severe diarrhea
- treated with vancomycin (not absorbed in GI tract so highly concentrated when it reaches colon) or metronidazole
- covers atypical pneumonia bugs
- 100x better absorbed by gram + bugs
- used for gram + mycoplasma
- used for gram - legionella and chlamydia
list the macrolide antibiotics
- erythromycin
- clarithromycin
- azithromycin
- roxithromycin
when do you use oxygen and external cooling
- used for treatment of severe respiratory diseases like pneumococcal pneumo
- used to treat respiratory syncytial virus in infants
respiratory syncytial virus
- causes respiratory infections
- contains F protein for formation of multinucleated giant cells (syncytial cells)
- lacks both HA and NA glycoproteins
- number 1 cause of penomonia in infants
what are coassociated medical diagnoses in patients with panic disorder?
- peptic ulcer disease
- hypertension
- major depression, alcohol abuse, simple phobias, and PTSD are frequently associated psychiatric diagnoses
what do you see in patients with stable angina on histology?
- if they don't die of an MI, you will see small patches of fibrosis and vacuolization of damaged myocytes in subendocardial locations
- caused by the gradual loss of myocytes
nl Na levels
135-145 mEq
what endocrine disorder can small cell carcinoma of the lung cause?
- can secrete ectopic ADH -> leads to SIADH
- increased total body water -> weight gain
- extra plasma water -> dilutional hyponatremia -> weakness, fatigue, and confusion
- urine sodium is increased -> inappropriately concentrated urine
what is used as second line MS treatment if TNF-b fails?
- glatiramer acetate
- a random copolymer of alanine, glutamic acid, lysine and tyrosine, which mimics myelin basic protein
- seems to induce changes in cell-mediated immunity
- muscle relaxant used to reduce muscle spasms associated with MS
- enhances Cell mediated immunity
- used in combination with 5-FU in treating colon cancers
- used for secondary progression MS
- suppresses cell mediated immunity, which is thought to reduce immune cell attack on the myelin sheath
- monoclonal antibody directed to specific adhesion molecules, thereby reducing leukocyte entry into the CNS
- used to treat MS
X-linked agammaglobulinemia
- failure of B cell precurors (pro and pre-B) to mature into B cells
- during nl B cell maturation, heavy chain genes are rearranged first, follow by LCs
- in XlinkedAG, maturation stops after HC rearrangement
- caused by mutation in B cell tyrosin kinase (Btk) -> failure to drive maturation
how does X-linked agammaglobulinema present?
- seen after 6 mo, when materanl Igs are depleted
- infection by H. inf, S. pneumo, S. aureus
- viral infections by enteroviruses and coxsackievirus
- absence of mature B cells
- underdeveloped germinal centers and peyers patches
Common variable immunodeficiency
- hypogammaglobulinemia, affecting only IgG or all classes
- diff from X-linked hypogammaglo-binlinemia b/c you have normal numbers of B cells
- B cells cannot differentiate into plasma cells though
- affects males and females equally
- hyperplastic lymph nodes
- onset of symptoms: childhood and adolescence
what are chylomicrons degraded by?
- chylomicrons are too large to enter cells, but are degraded in the circulation by lipoprotein lipase
- defect in lipoprotein lipase -> accumulation of chylomicrons in plasma
why might you get elevated VLDL?
- if pt eats high carb meal and has no lipoprotein lipase
- carb -> converted to fat in liver -> sent out in circulation as VLDL
- VLDL cannot be converted to LDL b/c there is no lipoprotein lipase
what serum abnormalities is caused by defect in lipoprotein lipase?
- can cause decrease in unesterified fatty acids, since chylomicrons contain esterified fatty acids
which nerve supplies the parotid gland?
- glossopharyngeal nerve (IX)
how does the corda tympani from the facial nerve (CN VII) control salvation?
- stimulates both submandibular and sublingual glands
what do golgi tendon organs do?
- sense tension within muscle and produce inhibitory feedback to alpha motor neurons to relieve tension
- alpha motor neruosn carry motor impulses in a reflex arc in response to afferent info from intrafusal fiber stretch via Ia nerves
extrafusal fibers
- constitute the bulk of the contracting muscle belly
- produce, but do not modulate muscle tension
- responsible for the power-generating component of muscle and are innervated by motor neurons.
intrafusal fibers
- sense muscle length
- in response to stretch, the produce afferent impulses via Ia fibers that stimulate a-motor neruons and muscle contraction
- muscle fibers that comprise the muscle spindle. These fibers are walled off from the rest of the muscle by a collagen sheath. This sheath has a spindle or "fusiform" shape, hence the name "intrafusal." While the intrafusal fibers are wrapped with sensory receptors
- acts as a calcium reservoir
how do you differentiate strep. pneumo from other a-hemolytic strep?
- sensitive to bile and bile-like compounds (e.g. optochin)
- the only virulence factor is the carbohydrate capsule
- need antibody to this capsule to overcome infection
- more than 80 capsule types; 23 have been put into a vaccine
- recommended for high risk groups (asplenic)
how do you clear Neisseria infections?
- alternative complement pathway
- individuals with C5-C8 deficiencies are at increased risk
what has a a-helical coiled fimbra?
- strep pyogenes (group A beta-hemolytic) has a fimbria made o an a-helically coiled M protein
- antibody against M protien will prevent infection
- however, raising antibodies to M protein can lead to rheumatic fever, so treatment by penicillin is used to prevent an ab response
what is the C carbohydrate used for in streptococci?
- an antigen of b-hemolytic strep
- used to divide the strep into groups: e.g strep pyogenes is group A
locked-in syndrome
- completely awake and cognitively intact with no abnormality of consciousness
- unable to move extremities and face b/c of lesions in brainstem- particularly the base of the pons
- produced by thrombotic stroke to the basilar artery
what symptoms do you get from interal carotid artery occlusion?
- should get collateral circulation from the other interanl carotid
middle cerebral artery strokes
- lateralizing symptoms: gaze deviation or hemiparesis
- MCA primarily perfuses the parietal cortex
- should get parietal lobe dysfunctions: agnosias, apraxias, hemi-neglect, language and calculation problems
posterior cerebral artery strokes
- PCA supplies the occipital lobe as well as medial portions of the parietal and temporal cortex
- symptoms should be laterlaizing, and visual changes are always present
superior cerebellar artery stroke
- ataxia and dysarthria
blocking antibody
- an IgG antibody against an allergen
- induced by administered small amounts of the allergen over a period of time
- when person is exposed to the allergen again, the IgG reacts with the allergen before it can reach the IgE coated mast cell
mech of antihistamine
- blocks histamine receptors
cromolyn sodium
- drug that stabilizes cell membranes
- inhibits degranulation, but would have no effect on allergen binding to IgE on mast cells
epinephrine and anaphylaxis
- increases intracellular cAMP -> decreases mast cell degranulation and causes sm muscle dilation of the airways
how is cAMP degraded in cells?
- degraded by phosphodiesterase
- Theophylline inihibits phosphodiesterase -> increases mast cell cAMP levels -> makes degranulation less likely
thymine dimers
- result when UV radiation causes a covalent linkage between two adjacent thymine nucleotides on the same strand
- impairment of nucleotide excision repair results in xeroderma pigmentosa: UV sensitivity, excessive freckling, skin cancers, CORNEAL ULCERATIONS
degenerative ataxia
- sign of ataxia-telangiectasia
- caused by defect in ATM (member of the PI-3 kinase family)
- involved in mitogenic signal transduction, detection of DNA damage, and cell cycle control
deficiency of adenosine deaminase (ADA)
- accumulation of deoxyadenosine and its derivatives, which are toxic to immature lymphocytes
hereditary nonpolyposis colorectal cancer
- caused by defect in mismatch repair -> development of colorectal cancer
- see microsatellite instability
how do you tell if a macrophage is phagocytically active?
- macrophages interact with a segment of the heavy chains of the IgG molecule (the Fc domain)
- macrophage receptors for IgG are called Fc receptors
- complement receptors are alos found on the surface
- phagocytosis occurs more readily when both components are present
where do you see collagenase?
- in eosinophils, mast cells, osteoclasts, and fibroblasts
nl K levels
3.5-5 mEq/L
nl BUN levels
- 7-18 mg/dL
nl creatinine levels
nl glucose levels
nl pCO2
33-45 mmHg
nl pO2
75-105 mmHg
describe esophageal innervation
- upper 2/3 contains striated muscle: derived from pharyneal arches and innervated by the vagus (CN X)
- lower 1/3 contains smooth muscle from splanchnic mesoderm: innervated by splanchinc plexus
what does the glossopharyngeal nerve (CN IX) do?
- taste
- swallowing
- salvation
- monitors activity of carotid body
when are lung volumes higher than normal?
- asthma
- brochospasm
- emphysema
- old age
- other conditions involving narrowed airways
which part of the brain is sensitive to hypoxic damage?
- hippocampus
- frequently damaged in anoxic brain injury
when do you see delerium tremors?
- hallucinations, agitation, and tremors
- seen in alcohol withdrawal
describe acanthosis nigrigans
- looks like a mole or wart, but actually due to epidermal hyperplasia
- can be seen in obesity, diabetes, and in patients with UNDERLYING cancers (adenocarcinomas of the chest or abdomen
define: hyperacusis
Inordinate sensitivity to sounds; it may be on an emotional or an organic basis.
what is the upper limit of nl 2-hour post prandial serum glucose?
what causes hyperglycemia?
- increased hepatic production of glucose
- diminished peripheral use of glucose
what does the citrate shuttle do?
- transports acetyl CoA from the mitochondria to the cytoplasm for fatty acid synthesis
- acetyl CoA combines with oxaloacetic acid in the mit to form citrate, and then is transported inot the cytoplasms
when do you see conversion of triglycerides into glycerol and fatty acids?
- in the presence of low insulin levels, or insulin insensitivity
ochronosis (alkaptonuria)
- reccesive
- lack of homogentisic oxidase -> blocks metabolism of phenylalanine-TYROSINE at the level of homogentistic acid -> accumulation of homogentisitic acid -> excreted in urine (black)
- can have depoisitin of pigment in the articular cartilage of joints -> arthritic disease
how does paroxysmal nocturnal hemoglobinuria present?
- associated with hemolytic anemia and reddish brown urine
- deficiency in phenylalanine hydroxylase (268)
- associated with mental retardation, elevated phenylalanine and hypopigmentation (less melanin)
the only muscle that can close the eye
- orbicularis oculi
- controlled by CN VII
- lesion in CN VII can also cause loss of corneal reflex (can't blink)
- ipsilateral paralysis of muscles of facial expression
- hyperacusis (increased sensitivity to sound, since CN VII controls the stapedius)
- lack of taste to anterior 2/3 of tonge (nevus intermedius)
- decreased lacrimation and salivation
what does CN III innervate?
- medial rectus, inferior rectus, superior and inferior oblique
what does CN VI innervate?
- lateral rectus
what does CN IV innervate?
- superior oblique
what is the most common cause of bronchiolitis and pneumo in children younger than 1?
- Respiratory syncitial viurs
- infection does not result in long lasting immunity
- reinfection can occur
- contains the F protein
- doesn't have either NA or HA glycoproteins
how do you prevent RSV infection?
- treat with palivizumab- monoclonal antibody against RSV that is produced by recombinant DNA
parainfluenza virus
- causes croup
- infectin of the larynx and other upper respiratory structures that occurs in children
- stridor (a wheezing sound)
- barking seal like cough
- single stranded - RNA
- HA and NA are part of same glycoprotein (not 2 diff spikes)
- F protein (fusion: cases infected host cells to fuse into multinucleated giant cells)
1. parainfluenza
2. Respiratory syncytial virus
3. mumps
4. measles
- affects lungs, kids, cause viremia
what happens to the O2 carrying capacity of the blood with decreased Hb concentration?
- decreased O2 carrying capacity
- each g of Hb can normally carry 1.34 g of O2
- does not affect O2 sat of Hb or the arterial PO2
what lesion is seen with wrist drop?
- radial nerve lesion
- radial nerve: 'great extensor nerve"
- innervates brachioradialis, externsors of wrist and fingers, supinator, and triceps (BEST)
- lesion: break in shaft of humerus
what causes bubonic plague?
- yersinia pestis
- transmission by animal (flea bite)
yersinia enterocolitica
- transferred by fecal-oral
- causes gastroenteritis
- caused by ingestion of contaminated foods: milk from farm aminals, or fecally contaminated water
- pain is in right lower quadrant- often confused for appendicitis
- can secrete enterotoxin
what can contaminated ground beef transmit?
- enterohemorrhagic E. coli (EHEC)
name an indirect agonist of norepi
- cocaine
- competitive alpha1 receptor blocker
- used to treat hypertension
- non-competitive alpha1 blocker
- used in cases of malignant hypertension and pheochromocytoma
- serotonin specific reuptake blocker
- also a weak alpha1 blocker (side effect: orthostatic hypotension)
- non-selective competitive alpha blocker
define: holoprosencephaly
A birth defect that occurs during the first few weeks of intrauterine life. HPE is a disorder in which the fetal brain does not grow forward and divide as it is supposed to during early pregnancy (incomplete cleavage of the embryonic forebrain/failure of the prosencephalon to cleave into the cerebral and lateral hemispheres). This brain malformation can range from mild to severe.
trisomy 13 (Patau syndrome)
- microphthalmia
- microcephaly and holoprosencephaly
- cleft lip and palate
- cardiac defects
- renal defects
- umbilical hernia
- rocker-bottom feet
trisomy 21 (Down syndrome)
- mental retardation
- epicanthic folds and flat facial profile
- simian creas
- umbilical hernia
- predisposition to ALL and AML
- hypotonia
- intestinal stenosis
- heart defects
- abundant neck skin
trisomy 18 (Edwards syndrome)
- prominent occiput
- mental retardation
- imcrognathia
- low set ears
- short neck
- loverlapping finters
- cogenital heart defects
- renal malformations
- limited hip abduction
- rocker bottom feet
define: porencephaly
Congenitally deficient development of the cerebral cortex and gray matter so that cystic cavities communicate with the brain surface.
define: anencephaly
- complete failure of higher brain structures to develop
- grossly deformed head with no cranial vault
define: encephalocele
Protrusion of the brain and meninges through a cranial fissure.
define: hydranencephaly
Complete or almost complete absence of the cerebral hemispheres. The space is filled with cerebrospinal fluid.
- an extreme form of proenchephay
- unlike anencephaly, the external head forms normally
name some other symptoms of Patau syndrome
- hyperconvex narrow fingernails
- scalp defects
- abnormal genitalia
- apneic spells
define: porencephay
- cyst or cavity in the brain that communicates with the ventricles
- occurs as a developmental anomaly or secondary to inflammatory disease or vascular accident
what should you think of when you see amyloid in the thyroid?
- medullary thyroid carcinoma
- carcinoma of the neuroendocrine parafollicular cells of the thyroid, rather than the follicular lining epithelium
- cells produce calcitonin -> form bands of amyloid
- is a component of MEN syndromes type IIa and IIb
MEN type IIa
- parathyroid disease
- pheochromocytoma
- medullary carcinoma of thyroid
MEN type IIb
- parathyroid disease
- pheochromocytoma
- medullary carcinoma
- mucocutaenous ganglioneuromas
- marfanoid habitus
medullary carcinoma
- amyloid from altered calcitonin molecules
- tumor of teh neuroendocrine parafollicular cells
how do you diagnose follicular thyroid carcinoma?
- tissue looks much like normal thyroid
- cannot be reliably diagnosed by fine needle aspiration alone
peritoneal carcinomatosis
- develops from transcoelomic spread of cancers located in the abdominal cavity
- most often source: ovarian cancer
- other cancers: carcinoma of the colon, can also cause disease
bronchogenic carcinoma- where does it metastasize?
- usually makes metastatic nodules within solid organs
carcinoid tumors- where do they metastasize?
- originate in GI tract (appendix or ileum)
- metastasizes to liver, and not to the peritoneum
gliobastoma multiforme
- most frequent malignant brain tumor
- made of anaplastic astrocytes
- does not metastasize outside of the CNS
- pseudopalisading of malignant nuclei
- often occurs in cerebral hemisphere
- prognosis is poor (1 year survival)
define: anaplastic
A term used to describe cancer cells that divide rapidly and bear little or no resemblance to normal cells.
what are the main etiologic associations with hepatocellualr carcinoma?
- chornic alcoholism
- food contaminants (aflatoxins)
- will see elvated serum a-fetoprotein
- invades the hepatic vein system and spreads first to the lungs
what makes aflatoxin?
- aspergillus flavus
- can cause hepatocellular carcinoma
what happens if you aspirate aspergillus?
- asthma
- type I hypersensitivity reaction
bacteria that act like fungi
- gram postiive rods
- actinomyces israelii
- nocardia asteroides
actinomyces israelii
- gram +, beaded, filamentous, anaerobic
- grows as normal flora in mouth and GI
- causes eroding abscesses (cervicofacial actinomycosis, abdomianl actinomycosis, and thoracic actinomycosis) folliwng trauma to the mucous membranes of the mouth or GI
- pus from the abscess shows yellow SULFER granules
- treat with genicillin G
Nocardia asteroides
- weakly gram _, partially acid fast beaded branching thin filaments
- frequently confused with TB b/c it's acid fast
- inhaled. grows in lungs -> abscesses and cavitations
- erosion into pleural space can occur
- treatment is a SNAP! Sulfa for Nocardia, actinomyces give penicillin
how do you treat the actinomyes and nocardia?
- it's a SNAP!
- sulfa for nocardia; actinomycese give penicillin
aspergillus fumigatus
- oppurtunisitic pathogen
- fungus balls (aspergilloma) are generally only seen in pre-existing cavities (bronchiectasis, TB), and not generally seen in tonsils
- produces aflatoxin
blastomyces dermatitidis
- respiratory pathogen that is seen as thick walled yeasts within granulomas
- caused by corynebacterium diptheriae
- forms a psuedomembrane in the pharynx
- releases an exotoxin that is damaging to the heart and neuronal cells
- don't scrape off the membrane! will cause bleeding and absorption of exotoxin
- culture on potassium tellurite agar and Loeffler coagulated blood serum
- difficulty extending thigh and flexing knee
- pain in posterior thigh, lateral leg and foot.
what does the femoral nerve innervate?
- ant compartment of thigh
- involved in extension at the knee
- damage: weakened hip flexion, loss of knee extension, sensory loss of ant. thigh, medial leg (sciatic supplies lateral leg), and foot
what does the inferior gluteal nerve innervate?
- inf gluteal nerve is a collateral nerve of the lumbosacral plexus
- originates from L5 - S2
- supplies the gluteus maximus
- damage: weakened hip extension, difficulty rising from seated position and in climbing stairs
what does the obturator nerve innervate?
- arises from L2 to L4
- supplies the anterior compartment of the thigh
- damage: weakened thigh adduction and medial roation
what does the superior gluteal nerve innervate?
- collateral of the lumbosacral plexus
- supplies the gluteus medius and minimus, and the tensor fasciae late
- damage: loss of abduction and gait impairment (Trandelenburg gait)
trandelenburg gait
an abnormal gait caused by weakness of the abductor muscles of the lower limb, gluteus medius and gluteus minimus. Patients with a lesion of superior gluteal nerve have weakness of abducting the thigh at the hip.
Duchenne muscular dystrophy
- most common form of muscular dystrophy
- X-linked. Seen in male children
- begins with weakness of proximal muscles
- deficiency in dystrophin caused by DNA coding frameshift -> formation of stop codon and total failure of dystrophin synthesis
- histology: variation in muscle fiber size, necrosis, fibrofatty replacement
- increased serum CK
how do you differentiate duchenne from becker's muscular dystrophy?
- duchenne is more severe and results from a frameshift mutation
- beckers: segmental deletions within dystrophin gene that does not cause frameshift
tell me more about the dystrophin gene
- found on Xp21
- one of the largest known genes of any species (2300 kB)
- contains 24 regions of 109 amino acids that are similar, but not identical repeats
- in women, the similarity of sequences can lead to misalignment of homologous material at meiotic synapsis -> frameshift mutation
which diseases result from expansion of a trinucleotide repeat?
- huntington disease
- fragile X
Fragile X
- major cause of mental retardation (second only to Down's syndrome)
- defect on long arm of the X chr causes breakage
- both males and females can be asypmtomatic carriers. they have an increased number of CGG tandem repeads in the familial metal reardation (FRM-1) gene
- sons with full mutations: bilateral macroorchidism
- malignancy of the biliary tree
- produces jaundice
- poor prognosis
- seen usually in patients with prior history of primary sclerosing cholangitis
describe the spleen
- red pulp: cords of billroth and spenic sinuses. has a red appearance. serves to filter blood
- white pulp: collections of lymphocytes aranged around a central artery in a configuration called the periaterial lymphatic sheath (PALS)
ito cells
- found in the hepatic perisunusoidal space, or in the space of Disse
- Ito cells are hepatic adipose cells and provide primary storage for vit A
Kupffer cells
- found in liver sinusoids
- belong to the mononuclear phagocytic system
- can breakdown damaged or senile RBCS, esp after splenectomy
define: adenxa
- tubes or ovaries
what is the most common predisposing factor in PID?
- infection by chlamydia trachomatis or N. gonorrhoeae
- other risk factors for ectopic pregnancy include prior ectopic pregnancy, exposure to DES, and induced abortion
- gram neg
- does not have a peptidoglycan layer and has no muramic acid
- found of columnar epithelial cells that line mucous membranes
- causes: conjunctivitis, cervicitis, and penumonia
- life cycle: elementary body (EB); Initial body
how do you differentiate staph epidermidis from other staphlococci?
- coagulase negative, non-hemolytic, and novobiacin sensitive
which bacteria does bacitracin resistance differentiate between?
- strep agalactiae from s. pyogenes (which is bacitracin sensitive)
which bacteria does catalase production allow you to differentiate?
- distinguishes the staphlococci from streptococcus
which bacteria does coagulase production help you differentiate between?
- staph aureus and yersinia pestis
what does optochin resistance help you differentiate between?
- major pathogenic alpha-hemolytic streptococci
- s. pneumo is optochin and bile sensitive
- s. viridans is resistant to both optochin and bile
what types of ciruclating antibodies to pts with type I DM have?
- anti-glutamic acid decarboxylase (anti-GAD) antibodies
- condition is linked to DR3, DR4, and DR3/4
- treat with insulin
what are ampehtamine salts used to treat?
and ADHD
- used to prevent clot formation in the outpatient setting
aka vit B1
- coenzyme needed for sever reactions
- required for thiamine deficiency (e.g. Wernicke's encephalopathy, and beriberi)
what is the hallmark of osteomalacia?
- widened OSTEOID seams due to poor mineralization of bone
- defective calcification of osteoid matrix
- although the trabeculae are normla in size, they don't mineralize effectively, and the rim of uncalcified osteoid is much larger than normal
- diffuse radiolucency
- when secondary to renal disease, osteomalacia is called renal osteodystrophy
why do you see increased numbers of osteoblasts in osteomalacia?
- trying to lay down more osteoid in an effort to strengthen the bone
- increased osteiod is not mineralized and bone remains soft and weak
when do you see increased osteoclast activity?
- hyperparathyroidism and Paget's disease
when do you see marrow fibrosis?
- hematopoietic disorders, and sometimes hyperparathyroidism
when do you see sparse trabeculae?
- in osteoporosis
describe mast cells
- uniform sized, spherical granules that contain materials in a scroll-like pattern
- several long microvilli, allowing for an increased number of binding sites for IgE
- nucleus has a lot of heterochromatin
- you should see collagen fibrils around the cell, since mast cells are found in connective tissue extracellular matrix
- connective tissue macrophages
- have heterogeneous granules of different sizes