physiology
Overview
Body compartments
principles
similar osmolality maintained because water moves
osmolality = 2[Na] + [glucose] + [BUN]
Tonicity = 2[Na] + [glucose]
these are the
effective osmoles
BUN doesn’t matter because it moves across membrane
Intraceulluar Fluid (66%)
high osmolality -> low ICF visa versa
Extracellular Fluid (33%)
interstitial (75%)
plasma (25%)
arterial
vascular
sodium content dependent
thirst
lightheadedness
paliptation
signs
orthostasis
>20 systolic pressure drop
>10 diastolic pressure drop
>20 pulse rate increase
decreased BP
low JVP
urine output
skin turgor
axillary sweat
dry mouth
regulators
RAS
sympathetic
natriuretic peptide
ADH
osmolarity sensor
lack of volume
volume trumps all
diagram
urine should reflect the body fluid
Sodium
filter a lot of sodium
resorb a lot of sodium
most control is in CCD
Potassium
rapid uptake into liver and muscle
insulin helps K+ absorption via Na/K ATPase
beta2 adrenergic stimulates potassium uptake when exercising, because muscles leak potassium
action potentials leave potassium extracellularly
H+ ions have variable effect depending on the anion of the acid
if the anion passes leaves cell, potassium will follow
mineral anions can pass
organics like lactic acids and ketoacids do not
distal tubule regulation (10% of K+ remains)
secretion matches potassium intake
increased urinary flow increases K+ secretion
more Ca++ absorption
aldosterone
increases sodium uptake and potassium secretion
sodium intake doesn’t matter
increased Na diet will increase distal tubule K wasting and decreased aldosterone K wasting
decreased Na diet will increase aldosterone K wasting, but decrease distal tubule K wasting
high K diet
leads to decreased AngII, leading to less Na uptake the DCT (more in collecting)
more aldosterone increases potassium channel opening
Calcium
in serum
50% bound
50% free
kidney reabsorption
Corrected Ca accounts for
albumin
pH
phosphorous
Other factors
PTH
mobilizes Ca from bones and kidney reabsorption
vitamin D affects absorption of Ca++
phosphate
diagram
regulation (high phosphate or low ca -> PTH released -> bone resorption of Ca and Phos and kidney excretion of Phos -> phos down, ca up)
PTH tells kidney to not resorb phosphate in proximal tubule
enhances uptake of phosphate from bones and blood
net drop
low levels stimulate bone resorption and prevent bone mineralization
FGF-23
needs co-factor Klotho
pH regulation
net
diagram
30 mmole intake from diet
40 mmols intake from metabolism
70 mmol excreted from urine
secretes protons by ɑ-intercalated cells in collecting duct (ammonium as the buffer/carrier) creating bicarb
ammonium reabsorbed in proximal and secreted in distal
buffers
ECF
bicarb (majority of buffering)
H+HCO3- <=> H2O + CO2
kidney makes bicarb
glutamine -> ammonium + bicarb in distal tubule
proximal tubule reabsorbs it
CO2 can leave via lungs
plasma proteins
phosphate
ICF
hemoglobin
protein
phosphate
Bone (40%)
bicarbonates released
Red Cell Mass regulation
oxygen sensor to signal EPO
Glomerulus
diagram
filtration
myoglobin >> hemoglobin >> albumin gets through
anionic drugs less filtered because of negative glycocaylyx and on podocytes
nephrin and podocin
GBM has heperan sulfate (negatively charged)
GFR
elimination of nitrogenous waste
oncotic pressure opposes filtration
hydrostatic pressure leads to filtration
systemic vascular pressure
regurgitation will oppose that
local afferent arteriole resistance
myogenic response
baroreceptors will constrict afferent if BP is too high or relax if BP is too low
local feedback control by ascending limb (tubuloglomerulo feedback)
macula dense cells of ascending limb send signals
detects Cl-
leads to vasoconstriction of afferent and renin release if there’s too much chlorine via adenosine
adenosine is vessels is a vasodilator
ultimately less GFR
efferent arteriole resistance
renin and AII constrict triggered by
sympathetics
myogenic response
macula densa
glomerulotubular balance
increase in filtration fraction leads to more absorption
increased capillary protein concentration (oncotic pressure)
Solute mass balance
amount filtered = GFR * concentration of solute
amount resorbed similar to amount secreted
total urine = Urine flow * concentration
tests
equation
C_plasma * GFR = C_urine * Q_urine
Clearance of x is flow of plasma that has been filtered of x
creatinine test
about 90% is GFR and 10% is secretion so creatinine clearance > GFR
but as GFR decreases, more percent is secretion
more creatinine concentration implies less GFR
collect urine over 24h
determine creatinine excreted in a day
PAH test (para-aminohippuric acid)
filtered and secreted 100%, so clearance is equal to effective renal plasma flow
BUN test (blood urea nitrogen)
some resorption, so GFR is more than clearance
hormones
constrictor
endothelin: both
adenosine: afferent
angII: efferent
catecholamines: afferent
dialator
PGE2:afferent
can prevent no GFR in crisis against alpha agonist
NO:afferent
proximal tubule
diagram
glucose is taken up greatly
inulin is not reabsorbed
reabsorb everything besides metabolism end products and excess water, salts (65%)
glucose and amino acids are absorbed 100%
control systems
loose epithelium
some sodium absorption via AII
channels
Na/H+ exchanger
results in bicarb absorption
loop of henle
25% resabsorption of sodium
diagram
concentration along loop
more sodium pumps near bottom of TAH (countercurrent multiplier)
filtrate is concentrated by water loss
ascending pumps out solute against a not huge gradient because it is already concentrated
very bottom doesn’t have pumps
concentration is explained by urea resorption through the medullary collecting duct
protecting vasa recta from stealing solute (countercurrent exchanger)
descending limb
thin ascending
thick ascending limb
sodium actively resorbed
transporters
apical.
ROMK
potassium secretion to build + charge
Na-K-2Cl
needs ROMK working to reabsorb sodium and chlorine
inhibited by furosemide and other loop diuretics
basolateral
NaK ATPase
Sodium resoprtion
Potassium secretion
CaSR
detects Ca/Mg and deactivates ROMK
leading to calcium wasting
Distal convoluted tubule
diagram
portion after macula densa
aldosterone -> Na resorption
Steep gradients and tight epithelium
fine regulation
thiazide diuretics
collecting duct
diagram
cortical collecting duct
medullary collecting duct
aldosterone -> Na resorption
K+ secretion
ADH
cAMP puts aquaporins in to recollect water
hypothalamic osmoreceptor
atrial stretch receptor (decreased stretch)
carotid baroreceptor (decreased)
occurs before you become thirsty
fine tuning
principal
resorb sodium
intercalated A
secrete H+
intercalated B
secrete bicarb
electrolyte pathology
hyponatremia
Diagnosis: <135 Na
overview
low urine sodium tells you there is low kidney circulation
check serum tonicity
[Na] can be low despite high [glucose]
proteins and lipids will take up volume that artificially decreases [Na] measurements
check GFR
low = acute injury or kidney failure
check urine osmol
low osmol means too much water intake
>100 mOsm/L indicates kidney malfunction
hypovolemic and hypervolemic can lead to decreased urine volume
check ADH due to effective volume decrease or something else
comprimised diluting segments
DCT acts solely on dilution via absorbing sodium
TAH absorbs sodium but it also leads to water absorption indirectly, thus not affecting tonicity
ADH presence
e.g. hypovolemia/hypotension
loss of salt like diarrhea
e.g. hyervolemia
CHF/cirrhosis can causes lack of perfusion in some places leading to ADH release
e.g. nausea, drugs, pain
e.g. Syndrome of Inappropriate ADH action (SIADH)
cancer of lung, or lung pathology
Renal cell carcinoma
Brain damage
symptom
increased ICF, ICP
seizing
chronically (3-4 days)
brain will normalize by extruding salt
don’t shrink it if it has been compensated
treatment
3% saline if symptomatic
restrict water intake if asymptomatic
restrict water intake
loop diuretics
Vaptan agents
give salty stuff to allow them pee
hypernatremia
diagnosis: >145 Na
access to water?
loss of water?
urine should be able to concentrate to 600 osm/L
symptoms
high osmolality => shrunken brain
cause
hypertonic ampules of bicarb given during a code
Kidney water loss
water diuresis (lack of ADH)
urine osmol < plasma osmol and total osmotic output is low
diabetes insipidus
central
pituitary problem
nephrogenic = ADH resistance
congeintal
drugs
osmotic diuresis
urine osmol > plasma osmol and total osmotic output is high
diabetes, DKA
urea
other water loss
burns
profuse sweating
GI loss
treatment
fix volume
fix water deficit
give enough water for ongoing losses
hyperkalemia
pseudohyperkalemia
potassium up due to K+ into ECF after blood transfusion
hemolysis of blood stored outside of body
fist clenching with a tourniquet
muscle used with tourniquet doesn’t allow K+ to be removed
thrombocytosis
if platelet count > 500k
K+ moves out of platelets during cloting
leukocytosis
centrifuging releases potassium so false positive elevated hyperkalemia
hereditary
diagnosis: [K+] > 5.1 mmol/L
check for oliguria and other cryptic causes
ECG
symptoms caused by resting potential elevation and closing of sodium channels
cardiac problems
muscle weakness and flaccid paralysis
metabolic acidosis
vasodilation
cause
decreased excretion
kidney injury
decreased GFR
must be significantly reduced
distal tubular Na+ reabsorption decrease
low effective blood volume
distal tubule flow
low effect blood volume
low aldosterone
hypoaldosteronism/addisons
increased cl reabsorption, retain K
hyporenin-hypoaldosteronism
diabetes
volume expansion
ARBs, ACEi
heparin has toxicity on adrenal zona glomerulosa
WNK4/WNK1 kinase
involved in AII signaling in DCT cell
spironolactone
transcelluar shift
insulinopenia
diabetic
beta blocker
fasting
somatostatin
cell breakdown
rhabdomyolysis
tumor lysis
hemolysis
increased ECF osmolality
h2o drags K+ with it
exercise
mineral acidosis
increased potassium intake (rare)
example of multiple linked things
treatment
urgent treatment if symptomatic
antagonize ECG effects with Calcium gluconate
stabilizes the heart
move K into cells
insulin
also give glucose to avoid hypoglycemia
b2 agonist
NaCO3
should make H+ leave cells and K+ enter
remove K
loop or thiazidediuretic
cation exchange resin
underlying cause
thiazide for Gordon’s syndrome
chronic
reduced dietary intake
hypokalemia
symptoms
lowered resting membrane, leading higher action potential threshhold
heart arrhythmias
rhabdomyolysis
breathing problems
ileus
Diagnosis
K/Creatinine ratio
high ratio implies loss from kidneys
creatinine is excreted in consistent amounts compared to water
cause
chronic renal loss of K
high aldosterone leads to K+ secretion
also leads to alkalosis
bushings, conns, licorice
liddle’s syndrome
diuretics
bartter’s syndrome
like loop diuretic
gitelmans syndrome
like thiazide diuretic
acute shift of K into ICF
insulin
epinepherine
aldosterone
alkalemia
H+ leaves cell, exchanging with K+
treatment
IV
PO
kcl
k citrate
k phos
acidosis/alkalosis
diagnosis
pH: 7.4
PCO2: 40 mm Hg
low indicates high CO2 clearance
HCO3-: 24 meq
deviation indicates metabolic problem
anion gap
unmeasured anions is largely due to albumin
expected anion gap = 4 albumin * 2.5 = 10 mmol
high anion gap implies too much acid
gap acidosis
delta gap = anion gap - expected anion gap
delta gap should equal bicarb loss
delta gap = delta bicarb
non-gap acidosis
check urine ammonium
low ammonium -> RTA
high ammonium -> diarrhea loss
if delta bicarb greater delta gap
non-gap acidosis in addition to gap
if delta bicarb is less than delta gap
metabolic alkalosis
osmolar gap
alcohol also contributes to osmolarity
metabolic
acidosis
cause
acid overproduction (anion gap>10)
lactic acidosis
A
anaerobic glycolysis due to low blood pressure or anemia
B
biochemical problem
thiamine deficiency
liver dysfunction
D
bacteria makes D-Lactate which is not metabolized
ketoacidosis
toxic alcohols
methanol
use fomepizole to block alcohol anhydrase from forming toxic metabolites
ethylene glycol
salicylate intoxication
salicylic acid damages cells leading to ketoacidosis and lactic acidosis
alkalization => salicylate more prevalent => does not cross
loss of bicarb (delta-delta = 0)
diarrhea
low ammonium excretion (delta-delta = 0)
renal tubular acidosis/uremia
try to figure urine ammonium
[Cl] - [Na] - [K] = cation gap
assume cations are due to ammonium if pH is low
proximal (type II)
defect in HCO3 reabsorption
e.g. carbonic anhydrase inhibitor
loss of bicarbonate
urine pH initially goes up and the goes down again when lots of bicarb is lost
distal (type I)
defect in acid excretion for alpha intercalated cell
urine pH is >5.5
can lead do potassium wasting
distal hyperkalemic (type IV)
low aldosterone
inhibits protons entering cells
cell thinks there is low proton content and so does not make ammonium
defect in acid excretion
compensation
in metabolic acidosis, pCO2 = [1-1.5] * HCO3
in metabolic alkalosis pCO2 = [.5-1]dBicarb
a higher pCO2 than expected means you are not getting rid of CO2
alkalosis
cause
vomitting
loss of stomach acid => more stomach acid production => leads to bicarb in serum
factors preventing bicarb loss
decreased GFR
increased aldosterone
hypokalemia
hypochloremia
maintenance of mineralocorticoid effect
diagnosis
urinary sodium and chloride low implies decreased GFR
high bicarb in urine causes sodium and water to be drawn out
treatment
treat the hypokalemia
respiratory
alkalosis
hyperventilation leads to low CO2
acute respiratory alkalosis compensation
HCO3 decreases 2 per 10 pCO2
chronic respiratory alkalosis (days) compensation
HCO3 decreases 5 per 10 PCO2 so pH is maintained
symptoms
cerebral vasospasm and ICP decrease
dizziness
acidosis
hypoventilation
decreased air intake or more dead space
acute
sedative drug overdose
acute exacerbations respiratory disorders
hypercapnia
chronic
sleep apnea
emphysema
acute compensation
HCO3 increases 1 per 10 pCO2
chronic (days) compensation
HCO3 increases 3.5 per 10 pCO2
symptoms
confusion
cerebral dilation and ICP increase
source
metabolism
food products
medications
intermediate products like lactic acid
hypercalcemia
diagnosis
symptom
weakness
coma
short QT
acute kidney injury
cause
pseudo: hyperalbumin
high PTH (PTH is cause)
hyperparathyroidism
FHH Thiazides (low urine Ca++)
kidney sensor doesn’t detect Ca++, so you make PTH and don’t pee out Ca++
no treatment needed
low PTH (other reason)
acidosis kicking ca off of albumin
malignancy
metastasis in bone
PTHrP releasing tumor (lung)
granulomatous disease
macrophages make calcitrol(1,25 vit D)
vitD/A intoxication
milk-alkali syndrome
lithium
thiazide
treatment
severe: volume repletion via IV NS
calcitonin
bisphosphonates
hypocalcemia
diagnosis
symptoms
trousseau sign
neuromuscular
strider
seizures
tetany
psychiatric disorders
CV
prolonged QT
arrhythmia
causes
pseudo
IV gadolinium
hypoalbuminemia
low PTH (PTH is the problem)
removal of parathyroid
hungry bone syndrome
hypoparathyroidism
primary
polyglandular synd
di-george
leads to high phosphate
high PTH (something else is the problem)
Vit D deiciency
lack of light
lack of intake
liver disease or kidney disease
they are involved in the metabolism
chronic kidney disease
tissue damage
leading to phosphate leak and binding to Ca
blood transfusions (citrate and EDTA bind to Ca)
alkalosis
increases Ca++ binding
resistance to PTH
deposition of Ca elsewhere
treatment
symptomatic: IV calcium
asymptomatic: oral vit D and Ca++ supplement
hypophosphatemia
cause
insulin
low phosphate diet (rare)
re-feeding syndrome
alkalosis (causes more glycolysis and consumption of phosphate)
reduction of cell ATP
phosphate is needed for glycolysis
magnesium
necessary for PTH release
hyper causes hypocalcemia
hypo causes hypocalcemia
pathology
diagnosis
urinalysis
color
red
microscopy
casts indicate renal origin
hematuria
RBC cast -> nephritis
pyuria
infection
epithelial cells
skin cells in urine is normal in the initial few drops
tubular version indicates glomerular damage
fat droplets
indicate protineuria and damage to cells
crystals
uric acid
calcium oxalate
mg ammonium phosphate
coffin lid
alkaline urine
electropheresis for proteinuria
<150 mg/day total
45% tamm hosftall
<30 mg/day albumin
albuminuria indicates foot process effacement
proteinuria without albuminuria could indicate lack of proximal tubule absorption or overload proteinuria
immunofluoresence
IgG,A,M heavy chain
kappa, lambda light chain
complement activation/deposition
kidney stones
diagnosis
flank pain
topiramate
calcium supplement, vitamind D/C
HT, obesity, diabetes, gout associated
symptoms
stone obstruction leads to pain
hematuria
fever
implies infection
emergency risk for sepsis
type of stones
calcium oxalate with a ca/phosphate core
calcium phosphate
uric acid
infectious (struvite)
bacteria makes urease => ammonia and alkaline urine
must remove stone because bacteria is protected by stone
cystine
could be due to aa transport defect
formation
randall’s plaques
Ca phosphate plaques on renal papilla
activity product ratio (concentration in a sense)
low urine volume (<1L)
high concentration of ca
urine calcium over >250 in men. >200 in women
95% idiopathic, mostly overweight white
maybe increased vit D absorption
bone resorption
renal excretion
high protein diet
acid load causes bone resorption
high sodium
more sodium is excreted, ca follows sodium
hyperparathyroidism
oxalic acid
fat malabsorption causes more oxalic acid
give calcium supplements
uric acid
usually due to insulin resistance
insulin resistance => less ammonia (buffer) => more acidic
uric acid precipitates
inhibitor
citrate (makes Ca more soluble)
metabolic acidosis leads to absorption of citric acid
less citrate => more stones
can be caused by lots of things
high calcium diet (paradoxical!?)
decreases oxalic acid absorption
BUT supplements can increase risk
treatment
diet
thiazide diuretic
lower calcium concentration
K-citrate supplement
glomerular disease
glomerulonephritis
nephritic syndrome presentation
hematuria
RBC cast
dysmorphic RBCs
glomerular proliferation on microscopy
focal vs diffuse (refers to population of glomeruli affected)
global vs segmental (refers to individual glomeruli)
hypertension
edema
there is a rapidly progressive glomerulonephritis version
increase in serum creatinine
summary
linear IF/anti-GBM ab
RPGN presentation
antibody to collagen IV in glomerular basement membrane
good pastures syndrome if this and pulmonary involvement
light
crescent
fibrinoid necrosis
treatment
steroid
plasmapheresis
granular IF/immune complexes
post-strep GN
Light: endocapillary proliferative GN
EM: sub-epithelial deposits
treatment: supportive
lupus nephritis
full house: IgG, IgM, IgA, kappa, lambda, complement
class determines treatment
immunosuppresants
steroids
IgA nephropathy
in asians and most common
mostly subclinical
synpharagytic
IgA vasculitis/Henoch Schonlein Purpura (HSP)
systemic IgA vasculitis presenting in child hood with colitis and skin rash
abnormal IgA results in abnormal complement and immune activation
EM: mesangial deposits of IgA
light: mesangial hypercellularity
treatment
ACEI/ARBs
steroids
pauci-immune IF
Anca diseases
antibody to neutrophils causes them to explode
MicroscopicPolyAngitis
GranulomatosisPA
EosinophilicGPA
LM-cresents
nephrotic (non inflammatory)
nephrotic syndrome
defined by proteinuria (>3.5 gm/d)
hypoalbuminemia
edema
hyperlipidemia
risk for
hypercoagulable
infection
atherogenesis
summary
normal (minimal change disease)
most common nephrotic cause in children
secondary causes:
Nsaids
focal segmental glomerulosclerosis
progresses to CKD
secondary causes:
low nephron mass
transplant: graft loss
membranous
PLA2R+ antibody
antibody to podocyte in a granular pattern
thickened
sub epithelial deposits
secondary causes
malignancy
infections
medications
lupus class V
other LM
amyloid
diabetic nephropathy
stages
prenephropathy
hyperfiltration
incipient nephropahty
non-detectable proteinuria
decreasing GFR
hypertrophy
overt nephropathy
>300mg albumin
>500mg protein
GFR decreases
kidney fibrosis
advanced nephropathy
low GFR
mesangial expansion = nodular glomerulosclerosis
pathophys
hyperglycemia
glycalation end products that damages the kidney
more oxidative stress
high kidney pressure
give ACEi to decrease glomerular pressure
hypertension
treatment
acei/arb
tubulo-interstital disease
ATN
toxins
hypersensitivity reaction: acute interstitial nephritis
much more interstitial space
eosinophils + other inflammatory cells
slow cumulative damage (analgesic abuse)
risk for acute papillary necrosis
direct toxicity (gentamycin)
contrast
rhabdo
metabolic
oxalate nephropathy
causes
autosomal recessive disease
fat malabsorption + vit c + oxalate foods
ethylene glycol poisoning
uric acid nephropathy
acute
rapid cell turnover
chronic
hyperuricemia
precipitation of crystals
nephrocalcinosis
hypercalcemia
staghorn calculus
infection
acute pyelonephritis (pelvis + nephrons)
gram negative rods in tubules and interstitium
UTI
reflux
diabetes
route can be hematogenous or ascending
chronic pyelonephritis
presentation
atrophic tubules (thyroidization)
scarring with distortion of calyces and pelvis
not necessarily infection related
types
chronic obstructive
secondary to reflux
polar scars
xanthogranulomatous pyelonephritis
immune disorders
sarcoidosis
granulomas for unknown reasons
post infectious immune activity
also lungs, LNs, heart, liver...
elevated ACE
IgG4 related disease`
infiltration of IgG4 plasma cells
whirling fibrosis
Crohns
neoplasia
multiple myeloma
cast nephropathy - light chain in tubules with giant cell reaction
amyloidosis (congo red)
cause
rheumatoid arthritis
multiple myeloma
vascular disease
Decreased renal blood flow
renal artery stenosis
FMD
atherosclerosis
atheroemboli
cholesterol embolus
infarction
wedge zone of coagulative necrosis
HTN
arterionephrosclerosis = HTN nephrosclerosis
dimpled shrunken kidneys
arteriolar hyalinosis
wrinkinling and global sclerosis
intimal fibrosis/sclerosis
malignant hypertensive nephropathy
flea bitten kidney with hemorrhages
fibrinoid necrosis
glomerular collapse
hemorrhage from vessel rupture
chronic:
hyperplastic arteriopathy (onion skin lesion)
scleroderma
fibrinoid necrosis
onion sknning (advanced intimal hyperplasia)
thrombotic microangiopathy
presentation
lowered haptoglobin
it binds to hemoglobin
endothelial injury
summary
classification
HUS
vessel wall damage
shiga-like toxin
thombocytopenia
kidney failure due to thrombosis of kidneys
atypical HUS
no diarrhea
caused by complement factor mutation, cancer, or auto immune
mostly children
TTP
fever
microangiopathic hemolytic anemia
thrombocytopenia
fluctuating neurological abrnomalities
renal disease
DIC
Pre-eclampsia
massive swelling of endothelial cells
subendothelial widening
acute kidney injury
diagnosis
decline in GFR over hours/days
increase Cr, available nephrons ~ 1/Cr
cause
Prerenal
presentation
RAAS, and higher creatinine
contraction alkalosis
reabsorb bicarb
low FENa < 1%
kidney is keeping salt appropriately
BUN/creatinine > 20:1
urea is reabsorbed in tubule
ECF volume depletion
low cardiac output
low systemic vascular resistance
increased renal vascular resistance
nsaids
Post renal (least common)
bilateral blockage
unilateral would be asymptomatic
Intrinsic
acute tubulointerstitial nephritis
focal loss of tubular epithelial cells -> urine sediment
ATN
paradoxical afferent vasoconstriction
increased chloride delivery to macula densa (less resorption)
increased Ca++
toxins
ischemia
epithelial cell injury
vascular effects
muddy brown casts
AIN (acute interstital nephritis)
WBCs
acute glomerulonephritis
proteinuria, dysmorphic RBC casts
acute vascular nephropathy
Sx
reversible, high mortality, could lead to CKD
ischemic damage
Rx
supportive
chronic kidney injury
causes
diabetes
kidney function declines with age
mechanical damage over time
oxidative stress, apoptosis, etc.
stages
summary
<60 ml/min/1.73 m^2 for more than 3 months
normal is 100
proteinuria incurs additional risk
end stage
kidney function needs replacement via dialysis or transplant
path
glomerulosclerosis
interstitial fibrosis
tubular atrophy
symptoms
CVD
stiffening of vessels from hyperphosphatemia
uremic toxins
inflammatory state
thrombogenic factors
less medication tolerance
secondary hyperparathyroidism
pathway
less vitamin d synth -> hypocalcemia
nephron loss -> can’t secrete phosphate -> FGF23 -> vit D suppression
PTH secretion
parathyroid enlargement, which tends to have less CaSR
hyperphosphatemia (binds free calcium)
usually low calcium despite high PTH
Sx
loss of bone
loss of bone in fingers
loss of distal third of clavicle
medial vascular and cardiac calcification
increased phosphate in cells results in changes that make them bone-like
stiffening of vessels leading to hypertension and increased afterload
worse form is calcific arteriopathy in which calficiation is in soft tissue
Uremic syndrome (symptomatic renal failure) [pericarditis, bleeding, encephalopathy]
GI
loss of appetite, particularly meat
metallic taste in mouth
uremic fetor (urea -> ammonium in oral cavity leading to ulcers
bleeding
anemia
normocytic hypoproliferative
lack of EPO
perhaps iron/folic acid deficiency or GI blood loss or bone problems
fatigue, dyspnea
dysfunctional platelets and lots of procedures with bleeding risk
Tx
frequent transfusion can lead to transplant rejection
recombinant EPO
DDAVP (vasopressin mimic)
release of wWF factor by endothelial cells
lipid
more TG because some not understood mechanism
decreased HDL
increased apolipoprotein-A1 due to less cleanse -> more atherosclerosis
statins (be careful of rhabdomyolysis)
sexual dysfunction
pregnancy
be careful of HTN
prematurity
get a transplant
metabolic acidosis
cannot excrete acid, loss of bicarb
bone disease
treatment
hyperparathyroidism
control hyperphosphatemia
low phosphate diet
phosphate binders
aluminum hydroxide
iron one
increase vit D
fear of increasing vascular calcification
suppress PTH
calcimimetic drugs
CaSR thinks there is calcium
expensive
surgical removal of parathyroid
proteinuria
ACEi/ARB to reduce protein filtration into urine
dialysis
definition
passage across membrane to remove solute and then put back desirable solutes
hemo
requires AV fistula or graft or catheter
peritoneal
use peritoneal space to hold the dialyzer fluid
transplant
candidate
low surgery risk and immunosuppression
life expectancy
immune respone
inate
adaptive
T cell
B cell
immune supression
T
B
steroids
hereditary
Autosomal Dominant Polycystic Kidney Disease
cause
PKD1 (85%) for polycystin 1 defect is more severe
sensor fluid movement
PKD2 (15%) for polycystic 2
ca channel regulator
cAMP in normally inhibited by PKD1/2
leads to cell division via RAS and cysts
two hit hypothesis as 1-5% tubules have cyst
treatment
Sx
cysts comprise 95% volume and grow very large
nausea
vomitting
trouble breathing
GFR decreases because it is pinched by cyst
HTN due to RAS activation
urinary stagnation -> stones
bleeding of cysts
impaired concentrating ability
can also have cysts in liver
mass effects like shortness of breath, early satiety
can also have MV prolapse, cerebral aneurysm, diverticulosis
cerebral aneurysm is most concerning
diagnose via ultrasound and also often HTN
treatment
mTOR inhibitor
vasopressin antagonist
Autosomal Recessibe PKD
cause
much rarer
fibrocystic PKHD1
Sx apparent early in life
significant liver dilitation in intrahepatic ducts
portal HTN
hepatic fibrosis
kidney cysts (smaller than ADPKD)
fetal oligohydraminos
big kidneys compress other things
clubbed features
altered facial features
low urine leads to less amniotic fluid
smaller lungs
stasis leads to infections in kidney and liver
Dx
poor differentiation between medulla and cortex
enlarged hepatic ducts
treatment
transplant
Alport
Cause
X-linked mutation of type IV collagen subunits
disruption of glomerular basement membrane
non uniform and interdigitating electron dense material
also eye
anterior lenticonus
also ear
hearing loss starting with higher pitches
treatment
transplant
RAAS blockade
APOL1
offers resistance to African Sleeping Sickness
increased kidney disease risk
particularly FSGS
tumors
RCC (lining of tubule)
presentation
smoker
hematuria
flank pain
renal mass
tumor can generate hormones
hypertension
polycythemia
hypercalcemia
staging (high chance of metastasis to bone or lungs)
types
clear cell (70%)
genetic version and sporadic
genetic = Von Hippel-Lindau syndrome
2 hit hypothesis
cells with out VHL think that they are hypoxic
kidney’s affected equally
clear (lipid) with web of capillaries
can turn into mesenchymal tissue (sarcomatoid change)
<1 yr expected survival
chromophobe
papillary
Urothelial Carcinoma
epi/presentation
exposed to carcinogens
male
painless hematuria
types
papillary
low grade
fusion of papilla
high grade
nuclear pleomorphism and random polarity of cells
probably loss 17p’s p53
flat (high grade)
urinary cytology
Nephroblastoma (Wilm’s Tumor)
malignant embryonal neoplasm -> divergent differentiation
WT-1 mutation
presentation
abdominal mass
2-5 y.o.
good prognosis (90% cure)
gross
solid/solitary mass that is delineated
rarely bilateral
cytology
diffuse anaplasia is bad
secondary hypertension
diagnosis
make sure it isn’t apparent RHTN (pseudo)
uncontrolled BP despite 3 meds, but controlled with 4
white coat htn
sodium
adherence to medications
don’t use BP raising drugs
nsaids
stims
cause
primary aldosteronism (conn’s)
presentation
HTN, hypokalemia, alkalosis
RAAS activation leads to aldosterone
renin, aldosterone levels
renal artery stenosis
Dx
rise in creatinine >30% after ACE/ARB
abdominal bruit
atherosclerosis
Tx: ACEi/ARB or angioplasty
FMD
distal lesions in women
Tx: angioplasty
pheochromocytoma (adrenal medulla)
diagnosis with metanephrines
resection and genetic testing
pharmacology
Diuretics
general
protein bound so does not go through GFR
require secretion through cells
mannitol
osmotic diuretic
can be used to reduce intracranial pressure and hypotension
acetazolamide
carbonic anhydrase inhibitor (Na/H)
treatment for
glaucoma
alkalosis
decrease bicarb resorption leading to increasing acidity
altitude sickness causes alkalosis because decreased pCO2
loop diuretic
inhibits Na/K/2Cl in TAH, thus impairing ability to dilute and concentrate (messes up medullary gradient) urine
leads to loss of Mg and Ca++
braking phenomenon after a few days
distal and collecting ducts start reabsorbing sodium
steady state of water and sodium is lower
used for edematous states
Sx
hypokalemia
ototoxitiy
thiazide
acts on DCT
inhibit NaCl resorption
Sx
hypertension
hypercalcemia
hyponatremia
K+ sparing
blocking ENAC in CD
corresponding block of ROMK leads to retention of K+
spironolactone and eplerenone