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143 Cards in this Set

  • Front
  • Back

Anterior Pituitary

- From Rathke's pouch


- FLAT PEG


-FSH, LH, ACTH, TSH, Prolactin, Endorphins GH



Posterior Pituitary

- From neuroectoderm (brain)


- Oxytocin, Vasopressin (ADH)

MEN1

PPP (3 Ps):


- Parathyroid tumors


- Pituitary tumors (Gh or prolactin)


- Pancreatic endocrine tumors (Zollinger-Ellison syndrome, insulinomas, etc.)




Autosomal dominant

MEN2a

MPP


- Medullary thyroid carcinoma (calcitonin)


- Parathyroid tumor (hyperplasia)


- Pheochromocytoma




ret gene mutation


Autosomal dominant

MEN2b

MPN


- Medullary thyroid carcinoma )calcitonin


- Pheochromocytoma


- Neuromas (oral/intentinal)




(+ marfanoid habitus)


ret gene mutation


Autosomal dominant

Basal cell carcinoma

Most common skin cancer


On sun-exposed areas


Low metastatic potential


Pearly pink nodules w/central depression/ulceration, rolled borders


Nests of busload cells and peripheral palisading of nuclei

Squamous cell carcinoma

Associated w/excessive sun exposure, immunosuppression, arsenic exposure


Locally invasive, may spread to LNs but rarely metastasizes


Ulcerative red lesions w/frequent scale. Keratin pearls


Actinic keratosis (scaly plaque) = precursor lesion

Akathesia

Subjective restlessness w/inability to sit still


Extrapyramidal side effect of antipsychotics


Treat by lowering antipsychotic dose or w/beta blockers

Scleroderma/CREST markers

CREST = anticentromere antibodies (in 40%)


Diffuse scleroderma = Anti-DNA topoisomerase I (scl-70) antibodies

snRNPs

"Snerps" = splicing done by splicosomes, made of snRNPs and proteins.


snRNPs made in nucleus


Antibodies vs. snRNPs in lupus

First area damaged during global cerebral schema?

Hippocampus!

Roles of sympathetic receptors

Alpha1: ↑ vascular SM contraction, ↑ pupillary dilator muscle contraction, ↑ intestinal and bladder sphincter muscle contraction


Alpha2: ↓ symp outflow, ↓ insulin release, lipolysis, ↑ platelet aggregation


Beta1: ↑ HR, contractility, renin release, lipolysis (1 heart)


Beta2: bronchodilation, vasodilation, ↑HR, contractility, lipolysis, insulin release, aqueous humour production. ↓ uterine tone, ciliary muscle relaxation (2 lungs)

Roles of parasympathetic receptors

M1: CNS, enteric NS (brain and bowels)


M2: ↓ HR, ↓ contractility of atria (heart)


M3: ↑ exocrine gland secretions, gut peristalsis, bladder contraction, bronchoconstriction, pupillary sphincter muscle contraction, cilia muscle contraction (everything else)

Roles of dopamine receptors

D1: Relaxes renal vascular SM


D2: Modulates transmitter release, esp in brain

Roles of histamine receptors

H1: ↑ nasal and bronchial mucus production, ↑ vascular permeability, contraction of bronchioles, pruritus and pain


H2: ↑ gastric acid secretion

Roles of vasopressin receptors

V1: ↑ vascular SM contraction


V2: ↑ H2O permeability and reabsorption in collecting tables of the kidney

Bethanechol

Direct cholinomimetic agonist (PS, M receptors)


Activates bowel and bladder SM (ileus, urinary retention)


Resistant to AChE


"Bethany chol (call) me if you can't pee or poop"

Carbachol

Direct cholinomimetic agonist (PS, M receptors)


"Carb"on copy of ACh


For: Glaucoma, pupillary constriction, relief of IOP

Pilocarbine

Direct cholinomimetic agonist (PS, M receptors)


Potent stimulator of sweat, tears and saliva (for Sjogrens). Also for open/closed angle glaucoma


Resistant to AChE


"Cry, drool, sweat on your "pilo"(carbine)"

Methacholine

Direct cholinomimetic agonist (PS, M receptors)


Challenge test for dx of asthma

Neostigmine

Indirect agonist of PS (M receptors) via anticholinesterase


↑ endogenous ACh


For: ileus/urinary retention, myasthenia grabis, reversal of neuromuscular junction blockade (rocuronium)


"Neo (No) CNS penetration"

Pyridostigmine

Indirect agonist of PS (M receptors) via anticholinesterase


For: myasthenia gravis


No CNS penetration

Physostigmine

Indirect agonist of PS (M receptors) via anticholinesterase


Anticholinergic toxicity (e.g. atropine)


Crosses BBB

Rivastigmine

Indirect agonist of PS (M receptors) via anticholinesterase


For: Alzheimer's

Edrophonium

Indirect agonist of PS (M receptors) via anticholinesterase


Historically for dx of myasthenia gravis

Atropine

Muscarinic antagonists (PS)


(also homatropine, tropicamide)


For: eye --> mydriasis and cycloplegia

Benztropine

Muscarinic antagonist (PS)


For: Parkinson's (↓ dopamine, ↑ ACh imbalance, blocks ACh cascade)


"Park my Benz"

Scopolamine

Muscarinic antagonist (PS)


For: motion sickness


"We all live in a yellow scopolamine (submarine)" = yellow barf, nauseous on a submarine

Glycopyrrolate

Muscarinic antagonist (PS)


For: pre-op, reducing airway secretions (if just oral use, for drooling or peptic ulcers)

Atropine

Muscarinic antagonist (PS) (so sympathetic unopposed)


For: bradycardia and ophthalmologic applications


↑ pupil dilation, ↓ secretions (airway, GI), ↓ gut motility, ↓ urgency in cystitis


Toxicity: ↑ body temp (due to ↓ sweating, rapid pulse, dry mouth, dry flushed skin --> "hot as a hare, dry as a bone, red as a beet, blind as a bat, mad as a hatter"

Epinephrine

Direct sympathomimetic


Beta>alpha


Anaphylaxis, asthma, hypotension


(alpha effects predominate at high doses)

Norepinephrine

Direct sympathomimetic


alpha1>alpha2>beta1


Hypotension

Isoproterenol

Direct sympathomimetic


beta1=beta2


electrophysiologic eval of tachyarrhythmias, can worsen ischemia

Dobutamine

Direct sympathomimetic


Beta1>beta2, alpha


Heart failure (ionotropic>chronotropic), cardiac stress testing

Phenylephrine

Direct sympathomimetic


alpha1>alpha2


Hypotension (vasoconstrictor), also ocular procedures, rhinitis

Albuterol (also salmeterol)

Direct sympathomimetic


beta2>beta1


albuterol for acute asthmal salmeterol for long-term asthma/COPD control

Clonidine

Sympatholytic (alpha2-agonist)


For: hypertensive emergency, ADHD, severe pain, some off-label uses

alpha-methyldopa

Sympatholytic (alpha2-agonist)


For: HTN in pregnancy

Phenoxybenzamine

Alpha-blocker (antagonist)


IRREVERSIBLE


Pre-op for pheochromocytoma

Phentolamine

Alpha-blocker (antagonist)


REVERSIBLE


If pts on MAO-inhibitors eat foods w/tyramine

Prazosin, terazosin, doxazosin, tamsulosin (-osin)

Alpha1-blocker (antagonist)


For: urinary sx of BPH

Mirtazapine

Alpha2-blocker (antagonist)


Depression

"-LOL"s

Beta blockers (antagonist)


For: angina, MI* (decr mortality), SVT, HTN, CHF* (slows progression), glaucoma


Avoid in cocaine users (risk of unopposed alpha-adrenergic receptor agonist activity), can mask hypoglycaemia in diabetics


Beta1 selective: Names from A-M (mostly)


Nonselective: Names from N-Z (mostly)

Eplerenone

Aldosterone antagonist, new


Used in place of Spironolactone (decreased side effects)


Can be used for Conn Syndrome (aldo-secreting adrenal tutor, leading to HTN, hypoK+, metabolic acidosis and depressed renin)

Tay Sachs

No Hexosaminidase A


Build-up of GM2 ganglioside


AR


"Tay SAX lacks heXosaminidase"

Niemann-Pick

No Sphingomyelinase


Build-up of sphingomyelin


AR


"No man picks (Niemann-Pick) his nose with his sphinger (sphingomyelinase)"

Gaucher

No Glucocerebrosidase (beta-glucosidase)


Build-up of Glucoceribroside


AR


Most common


Treated with enzyme replacement!


Gaucher = Glucocerebrosidase

Candida albicans

- Systemic or superficial infection: oral/esophageal thrush in immunocompromised, vulvovaginitis, diaper rash, endocarditis in IVDU, etc


- Germ tubes (true hyphae) @ 37C


- Yeast form (pseudohyphae) @ 20C


T-lymphos = prevent superficial infection


Neutrophils = prevent hematogenous spread


Rx: topical azole for vaginal, fluconazole/nystatin for oral/esophageal, fluconazole/AmphoB/Caspofungin for systemic

Aspergillus fumigatus

- Invasive aspergillosis (esp in immunocompromised and CGD)


- Allergic bronchopulmonary aspergillosis (if asthma/CF. May cause bronchiectasis and eosinophilia)


- Aspergillomas (in lung cavities, esp after TB infection)


Forms ring-enhancing brain lesions and paranasal sinus necrosis


Catalase +


NOT dimorphic


Form acute angles (less than 90°) and septations (A for Acute Angles)


Aflatoxins cause hepatocellular CA

Cryptococcus neoformans

Heavily encapsulated


Found in soil, pigeon droppings


Stains w/india ink and mucicarmine, although latex agglutination test is more specific


"soap bubble" lesions in brain

Mucor and Rhizopus

Disease mostly in ketoacidotic diabetic and leukaemia patients (like the ketones and glucose)


Penetrate cribiform plate and enter brain


Hyphae have wide angles (90°) and are non-septate

Histoplasmosis

Mississippi and Ohio River valleys (bats in caves)


Causes pneumonia


SMALLER than RBC (macrophage filled w/histo) ("histo hides in macros")


Rapid urine or serum antigen test

Blastomycosis

States east of Mississippi River and Central America


Causes inflammatory lung disease, can cause osteomyelitis


Granulomatous nodules


SAME SIZE as RBC ("Blasto has Broad-Based Budding")


Detect via urine antigen test

Coccidiomycosis

SW USA, Mexico, South America


Causes pneumonia and meningitis, can disseminate to bones and skin


↑ after earthquakes


Spherules filled w/endospores (much LARGER than RBC)

Paracoccidiomycosis

Latin America


Budding yeast w/"captain's wheel"


Much LARGER than RBC

Glioblastoma multiforme

Malignant, high grade tumor of ASTROCYTES


Most common primary malignant CNS tutor in adults


Can cross corrupt callous ("butterfly" glioma)


Pseudopalisading border w/central necrosis and hemorrhage

Meningioma

Common, usually benign


From arachnoid cells


Usually at convexities of hemispheres and parasagittal region, round lesion external to brain parenchyma


Presents w/seizures or focal neuro deficits


Resection/radiosurgery


Whorled pattern, psammoma bodies

Schwannoma

Benign tumor of Schwann cells


Usually at cerebropontine angle, often compresses CN VIII (loss of hearing and tinnitus)


S100+


Bilateral tumors in neurofibromatosis type 2

Hemangioblastoma

Associated w/von Hippel-Lindau


Often cerebellar

Pilocytic astrocytoma

#1 tumor in KIDS, usually in cerebellum


Benign tumor of astrocytes


Cystic lesion


GPAF+


Rosenthal fibres - eosinophilic, corkscrew fibres

Medulloblastoma

Highly malignant cerebellar tumor (primitive neuroectodermal tumor - cannot see in adults because neurons don't divide!)


can compress 4th ventricle causing hydrocephalus


"Drop mets" to spinal cord


Small round blue cells, Homer-Wright rosettes

Ependymoma

Highly malignant, ependymal cells


Usually in 4th ventricle, can cause hydrocephalus


Perivascular pseudorosettes

X-linked agammaglobulinemia

Bruton agammaglobulinemia (in Boys)


Defect in BTK gene = defective tyrosine kinase, no B cell maturation and NO Ig


Recurrent bacterial and enteroviral infections after 6 months (when maternal IgG decreases)


Absent/scant LNs, B markers present, but cells not mature

Common variable immunodeficiency

Defect in B cell differentiation


Acquired


↓ plasma cells (and Ig)

Selective IgA deficiency

Most common, most asymptomatic


Airway and GI infections, Atopy, Anaphylaxis to IgA containing products (e.g. blood)

Thymic aplasia

22q11 deletion, DiGeorge


Failure to develop 3rd and 4th pharyngeal pouches, absent thymus and parathyroids


↓ T cells, PTH, Ca2+


Recurrent viral/fungal infections

AD hyper IgE syndrome

aka Job syndrome


STAT3 mutation = decreased recruitment of neutrophils (due to deficiency of Th17 cells)


Coarse faces, cold staph abscesses (not inflamed), retained primary teeth, ↑ IgE, derm problems

SCID

Defective T and B cells


X-linked = defective IL-2


AR = adenosine deaminase deficiency


LOTS of infections (bubble boy)


Rx: bone marrow transplant

Ataxia-Telangiectasia

Defects in ATM gene = DNA double strand breaks and cell cycle arrest


Ataxia, spider angiomas and IgA deficiency

Hyper IgM

Class switching defect


defective CD40L on Th cells (cannot bind to B cells to tell them to switch classes!)


XR


Opportunistic infections early in life


↑ IgM


↓ everything else

LAD1

Impaired migration and chemotaxis of leukocytes


AR


Recurrent skin and mucosal infections, but NO pus, delayed umbilical cord separation (30+ days), impaired wound healing


↑ Neutrophils (because cannot bind to walls!)

Chediak-Higashi syndrome

Defect in lysosomal trafficking gene (LYST) = microtubule dysfunction in phagosome-lysosome fusion


AR


Partial albinism, peripheral neuropathy, neurodegeneration

CGD

Defect of NADPH oxidase = ↓ ROS and no respiratory burst in neutrophils


XR


Infection w/catalase + orgs


Nitroblue tetrazolim (NBT) dye reduction test is NEG; abnormal dihydrorhodamine test


n.b. if MPO deficiency, both tests will be normal

DNA laddering

Endonuclease cleavage into 180 bp fragments during karyorrhexis (apoptosis)

Intrinsic pathway apoptosis

- BAX and BAK = pro-apoptotic


- Bcl-2 = anti-apoptotic (Bcl-2 stabilizes mito membrane, prevents cytochrome c release [binds to/inhibits Apaf-1], cell lives = tumorgenesis)

Extrinsic pathway apoptosis

2 pathways:


- FasL binds to Fas receptor (CD95), activation of inactive caspases. Defective Fas-FasL = autoimmune disorders


- Cytotoxic T cell release of perforin and granzyme B

P-glycoprotein

aka MultiDrug Resistance Protein 1 (MDR1)


Expressed by some cancer cells to pump out toxins (incl chemotherapeutic agents)

Zero order elimination

Rate of elimination is constant regardless of target plasma concentration


PEA (looks like 0) = Phenytoin, Ethanol, Aspirin

Drug efficiency

Maximal effect a drug can produce

Drug potency

Amount of drug needed for a given effect (high affinity for receptor)

Bromocriptine

Dopamine agonist


Inhibits prolactin secretion (used for prolactinoma)

17α hydroxylase deficiency

Funnelled to ALDOSTERONE (glucocorticoids and androgens blocked)


HYPERtension, hypoK+, decr DHT


Presents as XY pseudohermaphroditism, XX lack of secondary sexual development

21-hydroxylase deficiency

Funnelled to ANDROGENS


HYPOtension, hyperK+, high renin activity


Most common


Salt-wasting in infancy, precocious puberty in childhood


XX virilization

11β-hydroxylase deficiency

Funnelled to ANDROGENS


HYPERtension (build-up of 11 deoxycorticosterone, so weak mineralocorticoid activity)


XX virilization



Neurofibromatosis type 1

von Recklinghausen disease


AD (100% penetrance, variable expression)


Mutations on NF1 gene on chromosome 17


Neurocutaneous disorder


Café-Au-Lait spots and cutaneous neurofibromas



Neurofibromatosis type 2

AD


Bilateral acoustic schwannomas at cerebropontine angle, juvenile cataracts, meningiomas, ependymomas


NF2 gene on chromosome 22

Tuberous sclerosis


AD


Neurocutaneous, multiorgan


Benign hamartomas

Universal enhancers of P450

Rifampin, phenytoin, and phenobarbital


Warfarin less effective

Universal inhibitors of P450

Cimetidine, amiodarone and TMP-SMX


Warfarin MORE effective!

Lesch-Nyhan syndrome

XR


Defective purine salvage due to decreased HGPRT (hypoxanthine-guanine phosphoribosyl transferase)


Hyperuricemia, Gout, Pissed off (aggressive, self-mutilation, Retarded, dysTonia)

Hypothalamus functions

TAN HATS


Thirst/H2O balance


Adenohypophysis control (anterior pit)


Neurohypophysis releases hormones from hypothalamus


Huncer


Autonomic regulation


Temperature regulation


Sexual urges


OVLT and area pastrami not protected by BBB

Thalamus

Major relay for all ascending sensory info except olfaction



Limbic system

5 Fs: Feeding, Fighting, Fleeing, Feeling and Sex


Hippocampus, amygdala, mammillary bodies, fornix, cingulate gyrus

Basal ganglia

Lentiform = Globus pallidus and putamen


Striatum = Putamen and caudate nucleus (lines 4th ventricle)


D1 = Direct, excitatory


D2 = Inhibitory pathway

Parkinson's

TRAPS (tremor [resting], rigidity, akinesia, postural instibility, shuffling gait)


Lack of dopamine (pallor of striatum nigra) = ↓ direct and ↑ indirect pathways


Presence of Lewy bodies (made of α-synuclein)

Huntington's

Huntingtin gene, chromosome 4


CAG nucleotide repeat


CAG = Caudate atrophy, ↓ ACh and GABA


Movement, aggression, dementia

Homunculus

(medial to lateral)


Foot, hand, mouth, tongue

Eyes direction in pathological conditions

Look TOWARDS a stroke (↓ inhibition)


Look AWAY from a seizure (↑ activity = ↑ inhibition

Fredrich Ataxia

Trinucleotide repeat disorder on chromosome 9 in gene for Frataxin (iron binding protein)


AR


Impairment in mito functioning leading to degeneration of multiple spinal cord tracts.


Staggering gait, falling, nystagmus, dysarthria, pets caves, hammer toes, hypertrophic cardiomyopathy


"Fredrich is your favourite FRAT brother, always STAGGERING and FALLING but he has a BIG HEART"



ACE inhibitor (names)

End in "-pril"


Captopril, enalapril, lisinopril, ramipril

Miosis

Constriction, parasympathetic


Neuron 1: Ediger-Westphal nucleus to ciliary ganglion via CN III (PNS fibres on outside, affected by compression; motor fibres on inside, affected by ischemia)


Neuron 2: short ciliary nerves to pupillary spincter

Mydriasis

Dilation, sympathetic


Neuron 1: Hypothalamus to C8-T2 (ciliospinal centre of bulge)


Neuron 2: Exit at T1 to superior cervical ganglion (along sympathetic chain near lung apex, subclavian vessels)


Neuron 3: plexus along internal carotid, through cavernous sinus, enters orbit as long ciliary nerve to pupillary dilator

CN III

Outer = PS fibres (constriction. If damaged, dilation)


Inner = motor

Alzheimer's

↑ risk in Down Syndrome (early onset: APP on chr 21)


Early onset: presenilin-1 (chr 14), presenilin-2 (chr 1)


Late onset: ApoE4 (Chr 19)


ApoE2 (Chr 19) is protective


↓↓ ACh


Senile plaques w/amyloid-β (synthesized w/amyloid precursor protein, APP); Neurofibrillary tangles (hyperphosphorylated tau protein)

Lewy body dementia

Dementia and visual hallucinations, then parkinsonian features


α-synuclein defect (like Parkinson's)

MS

(you know it, but the things you don't know...)


↑ protein in CSF (IgG)


Oligoclonal bands are diagnostic


** multiple white matter lesions separated in space and time**



Guillan Barre

(you know it, but the things you don't know...)


↑ CSF protein w/normal cell count (albuminocytologic dissociation). ↑ protein causes papilledema

Selegiline

For Parkinson's


Selectively inhibits MAO-B (preferentially metabolizes dopamine over norepinephrine and serotonin, thereby ↑ availability of dopamine)


(prevents dopamine degredation)

Alzheimer drugs

Memantine = NBDA receptor antagonist


Rivastigmine, galantamine, donepezil = AChE inhibitors

Stethoscope bell

Low pitched sounds


S1, S3, S4, low freq murmurs

Stethoscope diaphragm

High pitched sounds


S2, ejection sound, opening snap, mid-systolic click, high freq murmurs (most)

Paroxysmal Nocturnal Hematuria

Mutation of PIGA gene (involved in synthesis of glycosylphosphatidylinositol (GPI) - glycolipid needed to anchor several cell surface proteins)


CD55 and CD59 not anchored (normally inactivate complement and prevent MAC from attacking


Complement-mediated hemolysis


Thrombotic complications


Often presents w/pancytopenia and aplastic anemia


Dx w/flow cytometry

Inulin

= GFR; freely filtered and neither secreted nor absorbed


Cr clearance is approximate measure of GTR - slightly overestimates b/c moderately secreted by tubules

PAH (para-aminohippuric acid)

= effective renal plasma flow


both filtered and actively secreted in the proximal tubule (underestimates flow by 10%



Filtration fraction

GFR/Renal plasma flow


Inulin (or Cr clearance)/PAH clearance


Usually between 0.15-0.20

In kidney:


- ATII


- Loop diuretics


- Thiazides


- Aldosterone


- ADH


- Amiloride, triamterene



- ATII: (in PCT) stimulates Na+/H+ (contraction alkalosis)


- Loop diuretics: (in ascending loop) blocks Na+/K+/2Cl- reabsorption


- Thiazides: (in DCT) blocks Na+/Cl- reabsorption


- Aldosterone: (in CT) insertion of Na+ channel on lumen (reabsorption)


- ADH: (in CT) acts at V2 receptor, insertion of H2O aquaporins on lumen (reabsorption)


- Amiloride/Triamterene: (in CT) block ENaC (so less Na+ reabsorption and therefore less K+ leaving)

Renal tubule defects

Fanconi (PCT, wasting)


Bartter (Thick ascending loop, blocks Na+/K+/2Cl-, AR)


Gitelman (DCT, blocks NaCl, AR)


Liddle (DCT and CT, ↑ ENaC, AD)


"FaBulous Gold Liquid"

Angiotensin II

Vasoconstriction (vascular SM)


Constricts efferent arteriole


Aldosterone release (adrenals, to ↑ Na absorption) [volume]


ADH release (anterior pit, to ↑ H2O absorption)


↑ PCT Na+/H+ activity [osmolarity]


Stimulates hypothalamus (thirst)

RTA

Type 1 = DCT, intercalated cells can't secrete H+ (new HCO3- not generated); hypoK+


Type 2 = PCT, can't reabsorb HCO3-, instead excreted; hypoK+


Type 4 = Hypoaldo, aldo resistance, K+ sparing diuretics; hyperK+

Nephrotic

> 3.5 g protein/day, frothy urine, hyperlipidemia, fatty casts, edema, associated w/thromboembolism, ↑ risk of infection (loss of Ig)


"O F My Mom And Dad"


NephrOtic


- FSGS


- Minimal change


- Membranous


- Amyloidosis


- Diabetes

FSGS

Effacement of foot processes; focal and segmental sclerosis and hyalinosis


IF -


AAs and Hispanics, idopathic, HIV, sickle cell, heroin, etc.


Steroids = inconsistent response, may progress to chronic



Minimal change

Normal-looking glomeruli, IF-, effacement of foot processes on EM


CHILDREN ["mini"] (infection, immunization, immune stimulus, Hodgkin)


Only lose albumin


Steroids = excellent response

Membranous

Capillary and GBM thickening, GRANULAR deposits on IF (immune complex deposition, can be SLE), "spike and dome" sub-endothelial deposits


Idiopathic, drugs, infections, SLE, solid tutors


Steroids = poor response, risk of chronicity

Amyloidosis

Congo-red stain shows green birefringence ("Apple-oidosis")

Diabetic

Mesangial expansion, GBM thickening, Kimmelstiel-Wilson nodules (eosinophilic nodular glomerulosclerosis)


Non-enzymatic glycosylation of: 1) GBM = ↑ permeability and ↑ thickening, 2) efferent arterioles = ↑ GFR and mesangial expansion


ACE-Is slow progression of hyper filtration-induced damage

Membranoproliferative

Type I


- Subendothelial immune complex deposits, granular IF, "tram track" appearance (GBM splitting from mesangial growth)


- HBV, HCV, idiopathic (classic complement)


Type II


- Intramembranous immune complex deposits, "dense deposits"


- C3 nephritic factor

Nephritic

Inflammatory, hematuria and RBC casts in urine


Azotemia, oliguria, HN, proteinuria (<3.5 g/day)

Acute PSGN

Enlarged glomeruli, hypercellular


Granular appearance "starry sky" due to IgM, IgG and C3 deposition = subepithelial IC humps


Children 2-weeks post-GAS infection of pharynx or skin - M-protein virulence factor


Type III hypersensitivity reaction


Edema, coca-cola-coloured urine, HTN

RPGN

Crescent moons of fibrin and plasma proteins (C3b) w/glomerular parietal cells, monocytes and macros


Can be caused by:


- Goodpastures (TII hypersensitivity); antibodies to GBM and alveolar BM = linear IF


- Wegener's (c-ANCA)


- Microscopic polyangiitis (p-ANCA)

Diffuse proliferative GN

SLE or membranoproliferative GN


Wire-looping of capillaries, sub endothelial and sometimes intramembranous IgG-based IC w/ C3 deposition, granular IF

IgA nephropathy (Berger disease)

Mesangial proliferation, mesangial IC deposists, IgA-based IC deposits in mesangium


Seen w/Henoch-Schonlein purpura


Often w/mucosal infections (URI or acute gesture), Excess IgA gets stuck!


1-3 days later

Alport

Mutation in type 4 collagen


Thinning and splitting of BM


GN, deafness, sometimes eye problems

Kidney stones

- Calcium (80%) - radiopaque (can see); Ca oxalate or phosphate


- Ammonium magnesium phosphate (struvite) (15%) - radiopaque, infection w/urease+ bugs, staghorn calculi


- Uric acid (5%) - RadioLUCENT


- Cystine (1%) - radiopaque, cystinuria

Bladder cancer

Transitional cell carcinoma


- RFs: Smoking, cyclophosphamide, Aniline dyes, etc.


- Flat (early P53 mutations) or papillary (later P53 mutations)


Squamous cell carcinoma


- Chronic irritation


- RFs: schistosoma, chronic cystitis, smoking, chronic nephrolithiasis


Adenocarcinoma


- From urachal remnant, cystitis or extrophy

ATN

Granular "muddy brown" casts


Stage 1: inciting event


Stage 2: maintenance phase, oliguric; 1-3 weeks, risk of hyperK+, met acidosis


Stage 3: recovery phase: polyuric, BUN and Cr fall, risk of hypoK


↓ GFR, ↓ reabsorption of BUN (serum BUN:Cr < 15), ↓ reabsorption of Na (FENa > 2%), inability to [] urine (urine osm < 500 mOsm/kg)


Ischemic (PCT and thick ascending limb) or nephrotoxic (substances, crush, hemoglobulinuria) (PCT)

Structures perforating diaphragm

"I ate (8) 10 eggs at 12"


T8 = IVC


T10 = Esophagus + Vagus CN10


T12 = Aorta, thoracic duct, azygos vein

Virchow triad

Stasis


Hypercoagulability


Exposed endothelial collagen

Ratio for lung maturity in neonates

Lecithin:sphingomyelin


> 2 indicates lung maturity


<1.5 predictive of neonatal RDS


Tx: maternal steroids before birth, artificial surfactant for infant (O2 can cause retinopathy of prematurity and bronchopulmonary dysplasia)

ARDS

↑ alveolar capillary permeability (noncardiogenic pulmonary edema)


Formation if intra-alveolar hyaline membrane


Initial damage due to release of neutrophilic substances toxic to alveolar wall, activation of coal cascade and oxygen-derived free radicals

Collagen synthesis and formation

IN fibroblasts:


- Synthesis (Gly-X-Y, usually proline or lysine)


- Hydroxylation (+OH to proline/lysine, vit C needed. Scurvy)


- Glycosylation (pro collagen formed via hydrogen and disulfide bonds = triple helix. Osteogenesis imperfecta)


- Exocytosis


OUTSIDE fibroblasts


- Proteolytic processing (cleavage of disulfide rich terminal regions = insoluble tropocollagen)


- Cross-linking (reinforcement by cross-linkage to make fibrils. Elhers-Danlos)

Heteroplasmy

Presence of both normal and mutated tDNA (maternal DNA) resulting in variable expression in mitochondrial inherited disease

Vit Bs


1


2


3


5


6


7


9


12

1 = thiamine


2 = riboflavin


3 = niacin


5 = pantothenate


6 = pyridoxine


7 = biotin


9 = folic acid


12 = cobalamin

Scurvy

Swollen gums, bruising, hemarthrosis, anemia, poor wound healing, corkscrew hair


Cannot hydroxylate proline/lysine = defective collagen synthesis