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Step 1 - Neurology 3/Kidney 1
Neurology 3/Kidney 1
129
Medical
Graduate
03/30/2014

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Term
Alzheimers disease
Definition
Most common cause of dementia in the eldery
Inc risk in DS patients
*Early onset- APP (21), presenilin (14), presenilin-2 (1)
Late onset- ApoE4 (19)
ApoE2 is protective
Senile plaques- extracellular B-amyloid core > may lead to intracranial hemorrhages
Neurofibrillary tangles- intracellular abnormally phosphorylated tau protein (insoluble cytoskeletal elements)
Term
Picks disease
Definition
Dementia, aphasia, parkinsonian aspects, change in personality
Spares parietal lobe and post 2/3 of superior temporal gyrus
Pick Bodies- spherical tau protein aggregates frontotemporal atrophy
Term
Lewy-Body dementia
Definition
Parkinsonism with dementia and Hallucinations
*a-synuclein defect
Term
CJD
Definition
Rapidly progressive, weeks to months
Dementia with myoclonus
PrPc > PrPsc (B pleat resistant to proteases)
Term
Multiinfarct dementia
Definition
2nd most common cause in elderly
Syphilis, HIV, vitamins B1, B3, B12 deficiency, Wilsons dz, NPH
Term
Multiple Sclerosis
Definition
Autoimmune inflammation and demyelination of CNS
Pts present with optic neuritis (sudden loss of vision)
MLF syndrome (internuclear opthalmoplegia), hemiparesis, bladder/bowel incontinence
Charcot's triad: Scanning speech, intention tremor, Internuclear opthalmoplegia, and nystagmus
Term
Acute Inflammatory demyelinating polyradiculopathy
Definition
Most common variant of Guillan-Barre syndrome
*destroys schwann cells
Symmetric ascending muscle weakness/paralysis beginning in lower extremities; facial paralysis
Autonomic function may be greatly impaired > HTN, cardiac irregularities, hypotension
*Inc CSF protein with normal cell count
Assoc with Campylobacter jejune, and CMV
*Tx: plasmapheresis, and immune globulins
Term
Progressive Multifocal Leukoencephalopathy
Definition
Demyelination of the CNS due to destruction of the oligodendrocytes
Associated with JC virus
Seen in AIDS pts due to reativation
Rapidly fatal and progressive
Term
Acute disseminated encephalomyelitis
Definition
Multifocal perivenualr inflammation and demyelination after infection
Commonly measles or VZV, or certain vaccines like rabies or smallpox
Term
Metachromatic leukodystrophy
Definition
AR lysosomal storage disease
Arylsulfatase A deficiency
Buildup of sulfa tides leads to impaired production of myelin sheath
Term
Charcot Marie Tooth disease
Definition
Hereditary motor and sensory neuropathy (HMSN)
Defective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath
Term
Krabbe's disease
Definition
AR lysosomal storage disease due to deficiency of galactocerebrosidase
Buildup of galactocerebroside destroys myelin sheath
Term
Partial Seizures
Definition
Most commonly in the medial temporal lobe
Simple Partial- consciousness intact
Complex partial- impaired consciousness
Epilepsy- recurrent seizures
Status epilepticus- continuous seizure > 30 min
Term
Causes of seizures by age
Definition
Children- genetic, infection, trauma, congenital, metabolic
Adults- tumors, trauma, stroke, infection
Elderly- stroke, tumor, trauma, metabolic, infection
Term
Generalized seizures
Definition
Absence- (petit map) 3hz, no confusion, blank stare
Tonic clonic- alternating stiffening, and movement
Atonic- drop seizure, commonly mistaken for fainting
Term
Cluster Headache
Definition
Unilateral
Tx: oxygen and sumatriptan
Term
Peripheral vertigo
Definition
Most common type
Inner ear etiology;
positional testing > delayed hortizonatal nystagmus
Term
Central vertigo
Definition
Brain stem or cerebellar lesions
Directional change of nystagmus, skew deviation, diplopia, dysmetria
Positional testing > immediate nystagmus in any direciton
Term
Sturge Weber
Definition
Congenital disorder with port wine stain on face
Ipsilateral leptomeningeal angiomas, pheocromocytomas
*cause glaucoma, seizures, hemiparesis, MR
Sporadic
Term
Tuberous Sclerosis
Definition
HAMARTOMAS: Hamartomas in CNS and skin, Adenoma sebaceum, Mitral regurg, Ash leaf spots, cardiac Rhabdomyomas, Tuberous sclerosis, autosomal dOminant, MR, renal Angiomyolipomas, Seizures
Term
NF 1
Definition
Cafe Au Lait spots, Lisch nodules, optic gliomas
Pheochromocytomas
AD, 100% penetrant
Mutated NF1 gene on c17
Term
vonHippel Lindau syndrome
Definition
Cavernous hemangiomas in the skin, mucosa and bilateral RCC
Hemangioblastoma is retina, brain stem, cerebellum, pheocromocytomas
AD, mutated TSG VHL on c3
Term
Glioblastoma Multiforme
Definition
Most common primary brain tumor
*butterfly appearance, in cerebral hemispheres
<1 yr life expectancy
Stain for GFAP (astrocytes)
Pseudopalisading cells
Term
Meningioma
Definition
2nd most common primary brain tumor
In convexities of hemispheres
Arise from arachnoid cells, extra-axial
Typically benign and resectable, asymptomatic
*spindle cells concentrically arranged in a whorled pattern; psammoma bodies
Term
Schwannoma
Definition
3rd most common brain tumor
Often localized to CN 8
Resectable or treated with stereotactic radio surgery
S-100 positive
Bilateral found in NF-2
Term
Oligodendroglioma
Definition
Rare, slow growing
Frontal lobes; chicken wire capillary pattern
Fried eggs cells
Term
Pituitary adenoma
Definition
Most commonly prolactinoma
Bitemporal hemianopia due to inc pressure on optic chiasm
Term
Pilocytic astrocytoma
Definition
Well circumscribed
Often found in posterior fossa
My be supratentorial, GFAP pos
Benign, good prognosis
*Rosenthal fibers - eosinophilic, corkscrew fibers
Term
Adult Brain tumors
Definition
Good Men Straddle Open Pits
Term
Childhood brain tumors
Definition
Pre Mature Enfants Have Cough
Term
Medulloblastoma
Definition
Highly malignant cerebellar tumor
Primitive neuroectodermal tumor
Can compress the 4th ventricle, drop mets to spinal cord
*Homer wright rosettes - small blue cells, radiosensitive
Term
Ependymoma
Definition
Commonly found in the 4th ventricle
Can cause hydrocephalus
Poor prognosis
*Perivascular rosettes - rod shaped blepharoblasts near nuc
Term
Hemangioblastoma
Definition
Cerebellar, assoc with VHL when found with retinal hemangio
Can produce EPO > polycythemia
*foam cells
Term
Craniopharyngioma
Definition
Benign tumor, confused with pituitary adenoma
Bitemporal hemianopia
Most common childhood supratentorial tumor
*Rathke's pouch, calcification is common
Term
Epinephrine, Brimonidine
Definition
a2 agonists
Dec AH synthesis via vasoconstriction
Cause mydriasis, do not used in close angle
Blurry vision, ocular hyperemia
Term
Timolol, Betaxolol, cartelol
Definition
B blockers, dec AH synthesis
Term
Acetazolamide, mannitol
Definition
Dec AH synthesis via CA inhibition
Term
Pilocarpine, carbachol, physostigmine, echothiophate
Definition
Direct cholinomimetics (Pil and C), indirect (Phy and E)
Inc outflow via contraction of ciliary muscle and opening meshwork
Use Pilocarpine in emergencies
Cause mitosis and cyclospasm
Term
Latanoprost
Definition
PGF2a, inc outflow
Can darken color of iris
Term
Opiod analgesics
Definition
Morphine, etc.
Acts at opiod receptors to modulate synaptic transmission - open K channels, close Ca channels
Inhibits release of Ach, NE, 5HT, glutamate, substance P
Term
Butorphanol
Definition
Mu opiod partial agonist and kappa receptor agonist
Cause less respiratory depression than full opiod agonists
Can cause opiod withdrawal symptoms if pt is taking full opiod along with it
Term
Tramadol
Definition
Very weak opiod agonist also inhibits serotonin and NE reuptake
Decreases the seizure threshold
Term
Phenytoin
Definition
Inc Na ch inactivation
1st line for Tonic Clonic, SE prophylaxis
Also for Simple and complex
Gingival hyperplasia, hirsutism, megaloblastic anemia, teratogenesis, SLE syndrome, p450 induction, SJS
Term
Carbamazepine
Definition
Inc Na ch inactivation
Ist line: Simple, complex, tonic clonic
*1st line trigeminal neuralgia
Tox- diplopia, asplastic anemia, agranulocytosis, teratogenesis, SIADH, Stevens-Johnson syndrome
Term
Lamotrigene
Definition
Blocks VG Na channels
For Simple, Complex and TC
Tox- SJS
Term
Gabapentin
Definition
Inhibits VG Ca channels
For simple, complex, TC
Primarily used for peripheral neuropathy
Tox- sedation, ataxia
Term
Topiramate
Definition
Blocks Na ch, Inc GABA action
Simple, complex and TC
Used for migraine prevention
Tox- sedation, mental dulling, kidney stones
Term
Phenobarbitol
Definition
Inc GABAa action
Simple, complex and TC
*Ist line in children
Tox- Sedation, p450 induction
Term
Valproic acid
Definition
Inc Na channel inactivation, Inc GABA concentration
Ist line TC
Used for simple, complex, and Absence
Tox- heptatotox, NTDs, tremor, wt gain
Term
Ethosuxamide
Definition
*Blocks thalamic T type Ca channels
1st line for Absence
Term
Benzos
Definition
Inc GABA action
Ist line for SE acutely
Term
Tiagabine
Definition
Inhibits GABA reuptake
Simple, complex
Term
Vigabatrin
Definition
Irreversibly inhibits GABA transaminase > inc GABA
Simple, complex
Term
Levetiracetam
Definition
Unknown mechanism
Simple, complex and TC
Term
Barbituates
Definition
Facilitates GABA action by inc DURATION of Cl channel opening. Contrainidicated in porphyria
Resp and CV depression, CNS depression
OD tx is supportive
Term
Benzodiazepines
Definition
Facilitate GABA by inc frequency of Cl channel opening
Triazolam, oxazepam and midazolam are short acting > higher addictive potential
"Frenzos inc Frequency"
Anxiety, spasticity, SE, detox, hypnotic
Tox- less risk of OD than barbs, tx OD with flumazenil
Term
Non benzo hypnotics
Definition
Zolpidem, zaleplon, eszopiclone
Act via the BZ1 subtype of GABA receptor
Effects reversed by flumazenil
Term
Anesthetics gen pricinipals
Definition
Drugs with dec solubility in blood = rapid induction with recovery times
Drugs with inc solubility = inc potency = 1/MAC
Term
Inhaled anesthetics
Definition
Effects- myocardial depression, resp depression, inc cerebral blood flow
Tox- Hepatotox (halothane), Nephrotox (methoxy), Proconvulsant (enflurane), Malignant hyperthermia (all but NO)
Term
Thiopental
Definition
Barbituate IV anesthetic
High potency, high lipid solubility, rapid brain entry
Effect terminated by rapid redistribution into tissue
Term
Midazolam
Definition
Common for endoscopy
Severe post operative respiratory depression
Term
Ketamine
Definition
PCP analog Blocks NMDA receptors
Cause disorientation, halucinations, bad dreams
Inc cerebral blood flow
Term
Propofol
Definition
Rapid anesthesia induction, short term
Less post op nausea than thiopental
Potentiates GABA
Term
Local anestehtics
Definition
Esters- procainamide, cocaine, tertrcaine
Amides- Lidocaine, etc (Two Is)
Block Na channels, preferentially activated
Given with epinephrine to enhance effects (vasoconstriction)
In infected tissue, alkaline don't work as well, use more
First blocks small, then myelinated
Term
Succinylcholine
Definition
Depolarizing NMJ blocker
Strong Ach receptor agonist - sustained depot and prevents contraction
Antidote for phase 2 is neostigmine
Term
Non depolarizing NMJ
Definition
Tubocurarine, atracurium, mivacurium, pancuronium, vecuronium, rocuronium
Competitive antagonists - compete with Ach for receptors
Reversal- neostigmine, edrophonium
Term
Dantrolene
Definition
Prevents release of Ca from sarcoplasmic reticulum
Tx of malignant hyperthermia, NMS
Term
Dopamine agonists (Alz)
Definition
Bromocriptine, (ergots) pramipexole, ropinirole (non ergot)
Term
Inc dopamine (alz)
Definition
Amantadine (also anti-influenza)
L dopa/carbidopa
Term
Prevent breakdown (alz)
Definition
Selegilie (selective MAO type B inhibtor)
Entacapone, Tolcapone (COMT inhibitors)
Term
Curb excess cholinergic activity (alz)
Definition
Benztropine - antimuscarinic, impoves akinesia rigidity
Term
L dopa/Carbidopa
Definition
Inc level of DA in brain
Carbidopa is a peripheral dopa decarboxylase inhibitor
Tox- arrythmias from increased peripheral catecholamines
Term
Selegiline
Definition
MAO-I B, metabolizes DA over NE and 5HT
Adjuntive agents to Ldopa in tx of parkinsons
May enhance effects of L dopa
Term
Memantine
Definition
NMDA R blocker for alzheimers
Help prevent excitotoxicity
Term
Donepezil, galantamine, rivastigmine
Definition
AchE inhibitors
Term
Huntington's drugs
Definition
Tetrabenzine, and reserpine - inhibit VMAT
Limit DA vesicel packaging and release
Haloperidol - DA R antagonist
Term
Sumatriptan
Definition
Serotonin agaonist
Inhibits trigeminal nerve activation,
Vasoconstriction
Term
Kidney Embryo
Definition
Mesonephros- interem kidney, becomes male genital system
Metanephros- Appears in 5th week, gives rise to:
- Ureteric bud: caudal end > ureter, pelvises, calyces, and CD
- metanephric mesenchyme > glomeruli through to DCT
Ureteropelvic junction- last to canalize, common site of obstruction
Term
Potters syndrome
Definition
Oligohydramnios > compression of the fetus, limb deformities, facial deformities, and pulmonary hypoplasia
Causes: ARPKD, post urethral valves, bilateral renal agenesis
Term
Horseshoe kidney
Definition
Trapped on the IMA
Assoc Turners syndrome
Term
Multicystic dysplastic kidney dz
Definition
Abnormal interaction between ureteric bud and metanephric mesenchyme > nonfunctional kidney consisting of cysts and CT
Uni- compensatory increase of good kidney, asymptomatic
Term
Ureters course
Definition
Pass under the uterine artery and under ductus deferens
"water under the bridge"
Term
Fluid compartments
Definition
"hikin" = high K+ intracellular
60-40-20 rule
-60% total body water
-40% ICF
-20%ECF > 15% interstitial volume, 5% plasma volume
Term
GFR barrier
Definition
Fused BM with heparin sulfate (neg charge barrier)
Charge barrier is lost in nephrotic syndrome resulting in albuminuria, hypoproteinemia, generalized edema and hyperlipidemia
Term
Renal Clearance
Definition
C = UV/P
If C < GFR then there is tubular resorption
If C > GFR then there is tubular secretion
Term
GFR
Definition
Inulin clearance = GFR
GFR = UV/P
Normal GFR is around 100
Cr Cl is a more appropriate estimate of GFR
Term
Effective Renal Plasma Flow
Definition
PAH CL = EPRF, both filtered and actively secreted substance
RBF = RPF/1-Hct
Term
Filtration Fraction
Definition
GFR/RPF
Normal is 20%
Filtered Load = GFR x plasma concentration
Term
Drug effects on clearance
Definition
NSAIDs block PGs which normally dilation afferent arteriole
Inc RPF, Inc GFR, so FF is the same

ACEI block AG2 which normally constricts efferent arteriole leading to dec RPF, Inc GFR, so inc FF
Term
Changes in glomerular dynamics
Definition
Aff con- Dec RPF, dec GFR, NC FF
Eff con- Dec RPF, Inc GFR, Inc FF
Inc plasma protein- NC RPF, dec GFR, dec FF
Constric ureter- NC RPF, dec GFR, dec FF
Term
Reabsorption and secretion calculations
Definition
Filtered load= GFR x P
Excretion rate= V x U (urine flow rate x conc of urine)
Reabsorbed= filtered - excreted
Secreted= excreted - filtered
Term
Glucose clearance
Definition
Glucose normally completely reabsorbed by PCT by Na/glucose transporter
At 160, glycosuria begins
At 350, all transporters are completely saturated
Normal pregnancy has glycosuria and aminoaciduria
Term
Amino Acid clearance
Definition
Na dep transporters in PCT reabsorb aa
Hartnups dz- def in tryptophan transporter, leads to pellagra
Term
PCT
Definition
ALL- glucose, AA
Most- HCO3, sodium, chloride, phosphate and water
Secretes- ammonia
PTH blocks Na/PO3 transporter (PO3 excretion)
AT2 stim Na/H exchange (Inc Na, H2O and HCO3 resorb)
Term
Thin descending loop of Henle
Definition
Passively resorbs water via hypertonicity in medulla
*Impermeable to sodium
"Concentrating segment" > hypertonic urine
Term
Thick ascending loop of Henle
Definition
Actively resorbs Na, K and Cl
Induces paracellular resorption of Mg and Ca through pos lumen potential, gen by K back leak
*impermeable to water
Term
DCT
Definition
Actively resorbs Na, Cl
PTH inc Ca/Na exchange (Inc Ca resorption)
Term
CD
Definition
Resorb Na in exchange for secreting K and H
Aldosterone acts on R, insert Na ch on luminal side
ADH acts at V2 R, insert aquaporin on luminal side
Term
PCT transporters
Definition
Na/Glucose cotransport (in)
Na(in)/H(out) antiporter
Cl(in)/base(out) anti porter
H binds HCO3, breaks down to H2O and CO2 by CA, absorbed, combined to H2CO3 by CA and dissociates into H and HCO3 again. HCO3 resorbed into blood
Na(out)/K(in) ATPase on interstitial side
Term
Thick ascending LOH transporters
Definition
Na/K/2Cl cotransport into cell from lumen
K back leak channel into lumen
Mg/Ca passive diffusion
K, Cl diffuse down cellular gradient into blood
Na/K ATPase into blood
Term
DCT transporters
Definition
Na/Cl cotransport into cell
Ca diffusion into cell
Na/K ATPase on interstitial side
Na(in)/Ca(out) anti porter on interstitial side
Cl diffusion
Term
CD
Definition
Na diffusion
K back leak channel
H2O diffusion via aquaporins
Intercalated cells: H ATPase into lumen, HCO3(out)/Cl(in) interstitial side
Term
Concentrations along PCT
Definition
Na, K, Cl and urea are all resorbed slower than water
Pi, HCO3, AA and glucose are all resorbed faster than water
Term
RAAS system
Definition
Renin is stim by Dec BP (JG cells), Dec Na del (MD cells), inc symp tone (B R)
Angiotensinogen from liver is converted to AT1 by renin
AT1 is converted to AT2 by ACE from lungs
ACE blocks bradykinin
Term
Effects of AT2
Definition
Vasoconstriction by the AT1 R on vasc Sm Mm (inc BP)
Constrict eff arteriole > inc FF to preserve renal function, with compensatory Na resorption in PCT
Aldosterone from adrenal glands *
ADH from post pit, inc water channel insertion on prin cells
Inc PCT Na/H activity
Stim hypothalamus > thirst
Affects baroreceptors to limit reflex bradycardia > preserves both BP and blood volume
Term
Aldosterone effects
Definition
Secreted in response to Inc K and AT2
Inc Na Ch and Na/K pump insertion in principal cells
Enhance K and H secretion
*creates favorable gradient to resorb Na and H2O
Term
ANP
Definition
Released from atria in response to stretch
Relaxes vasc smooth muscle via cGMP causing inc CFR, dec renin
Acts at PCT to inc Na filtration and GFR
*no compensatory Na resorption
Term
ADH
Definition
Primarily regulates osmolarity, but also responds to low blood volume
Term
JGA
Definition
JG cells- modified Sm mm of the afferent arteriole
MD- NaCl sensor, part of DCT
JG cells secrete renin in response to low BP, low NaCl
*B blockers can decrease BP by blocking B1 R of the JGA causing dec renin release
Term
Erythropoietin
Definition
Released by interstitial cells in the peritubular capillary bed in response to hypoxia
Term
1,25 OH vitamin D
Definition
Proximal tubule cells convert vit D via 1a hydroxylase (stim by PTH)
Term
Prostaglandins
Definition
Paracrine secretion vasodilates the afferent arterioles to Inc GFR
Blocked by NSAIDs (leads to acute renal failure)
Term
Hormones acting on the Kidney
Definition
Term
PTH
Definition
Secreted in response to dec plasma Ca and inc plasma PO4
Causes inc Ca resorption, inc vit D production
Causes inc Ca AND PO4 absorption from gut
Acts in PCT and ThAscLimb LOH
Term
Potassium Shifts
Definition
Out of cell: digitalis, hyperosmolarity, insulin def (DKA), lysis of cells, acidosis, B-adrenergic antagonist
In to cell: hypoosmolarity, insulin (inc Na/K ATPase), alkalosis, B-adrenergic agonists (Inc Na/K ATPase)
Term
Na
Definition
Low: nausa, malaise, stupor
High: irritability, stupor, coma
Term
K
Definition
Low: *U waves on ECG, flat T waves, arrythmias, Mm weakness
High: *Wide QRS and peaked T waves, arrythmias, Mm weakness
Term
Ca
Definition
Low: Tetany, seizures
High: Stones, bones, groans, psychic moans
Term
Mg
Definition
Low: Tetany, arrythmias
High: *Dec DTRs, lethargy, bradycardia, hypotension, cardiac arrest, hypocalcemia
Term
PO4-3
Definition
Low: bones loss, osteomalacia
High: Renal stones, metstatic calcifications
Term
Acid Base Phys
Definition
Met Acid- Dec pH, dec Pco2, Dec HCO3 (hypervent to comp)
Met Alk- Inc pH, Inc Pco2, Inc HCO3 (hypovent)
Resp Acid- Dec pH, inc Pco2, Inc HCO3 (inc renal HCO3 res)
Resp Alk- Inc pH, dec Pco2, dec HCO3 (dec renal HCO3 res)
Term
Respiratory Acidosis
Definition
Hypoventilation (not blowing off CO2)
Acute/Chronic lung disease
Opiods, sedatives
Weakening of respiratory muscles
Term
Metabolic acidosis - Anion Gap
Definition
MUDPILES:
Methanol
Uremia
DKA
Propylene glycol
Iron tables, INH
Lactic acidosis
Ethylene glycol
Salicylates
Term
Metabolic acidosis - Nonanion gap
Definition
HARD-ASS
Hyperalimentation
Addisons dz
Renal tubular acidosis
Diarrhea
Acetazolamide
Spironolactone
Saline infusion
Term
Respiratory alkalosis
Definition
Hyperventilation (high altitude exposure)
Salicylates (early)
Term
Metabolic alklaosis
Definition
Loops diuretics
Vomiting
Antacid use
Hyperaldosteronism
Term
Type 1 RTA (distal)
Definition
Defect in CD ability to excrete H+
Untreated patients have a urine pH < 5.5
Assoc with hypokalemia
Inc risk for calcium pyrophosphate stones
Term
Type 2 RTA (proximal)
Definition
Defect in proximal tubule HCO3 reabsorption
Seen in Fanconiis anemia
Urine pH < 5.5, assoc with hypokalemia
Inc risk of hypophosphatemic rickets
Term
Type 4 RTA (hyperkalemic)
Definition
Hypoaldosteronism or lack of CD response to aldosterone
Hyperkalemia impairs ammoniagenesis in the PCT
Dec buffering capacity and dec Urine pH
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