GN 2 Flashcards
nephrotic syndrome hallmark clinical findings
proteinuria >3g/day
peripheral edema
hypoalbuminuria
(may have just the proteinuria)
etiologies of nephrotic syndrome
1/3 = systemic disease with renal manifestations
2/3 = idiopathic
systemic diseases causing nephrotic syndrome
amyloidosis
diabetic nephropathy
HIV nephropathy
SLE
hep C
types of idiopathic nephrotic syndrome
minimal change dz
membranous nephropathy
focal segmental glomerulosclerosis
amyloidosis nephrotic syndrome
associated with multiple myeloma (AL) or RA, IBD, and chronic infection (AA)
misfolded non-fxnl proteins
diabetic nephropathy
MC in type I
DM pts are prone to other renal dz (papillary necrosis and nephritis)
nephrotic syndrome s/s (6)
peripheral edema
abdominal fullness
dyspnea
increased infection
hypercoagulability
protein calorie malnutrition
peripheral edema nephrotic syndrome
LOW serum albumin causes drop in oncotic pressure shift to 3rd space
this also causes abdominal fullness (ascites) and dyspnea
dyspnea nephrotic syndrome
due to pulmonary edema, pleural effusion, ascites on diaphragm
infection nephrotic syndrome
due to urinary loss of Ig and complement
also have increased susceptibility to pneumococcus
hypercoagulability
increased likelihood of arterial and venous thromboemoboli
particular FVT and renal vein thrombosis
renal vein thrombosis - be sure to distinguish from stone
protein calorie malnutrition
negative protein balance
more is being excreted than is being taken in
catabolic state may be masked by weight gain from edema
causes vitamin deficiencies (loss of binding proteins)
lab derangements off nephrotic syndrome
decreased serum albumin
hyperlipidemia
vitamin deficiencies
elevated ESR (increased fibrinogen)
hypocalcemia
U/A - proteinuria and oval fat bodies
hyperlipidemia nephrotic syndrome
hepatic production of lipids increases to maintain oncotic pressure despite losing so much protein in urine
decreased VLDL clearance
inflammation also exists - accelerated CAD
hypocalcemia why in nephrotic syndrome
vitamin D deficiency
tests used to elucidate etiology of nephrotic syndrome
ANA
C3/C4/complement
SPEP/UPEP
infectious dz work up
renal biopsy
general management of nephrotic syndrome
Diet and Edema control
hyperlipidemia management
anticoagulation
diet and edema nephrotic syndrome
SALT restriction
diuretics (loop and TZD will be higher doses)
hyperlipidemia management nephrotic syndrome
diet and exercise don’t help
aggressive pharmacotherapy
hypercoaguable state nephrotic syndrome
prone to membranous glomerulopathy and Afib
anticoagulant 3-6 months for thrombosis or until dz is controlled
consider prophylactic anticoagulation
minimal change dz (Nil Dz)
mc in children, can affect adults
adults: M=F, children: M>F
may be idiopathic, follow URI, or paraneoplastic syndrome (Hodgkin), associated with drugs (NSAID)
manifestations of Nil Dz
susceptible to infection
tendency to thromboembolic events
CAN cause acute renal failure
severe hyperlipidemia, protein malnutrition
Nil Dz renal biopsy
tissue looks almost completely normal
little disposition of immune complexes
minimal change disease tx
prednisone 1mg/kg/day
adults longer
some patients become resistant to corticosteroid
majority of patients will relapse and req steroid again - ESRD progression rare
membranous nephropathy etiology
common cause of nephrotic syndrome in adults (idiopathic)
in children usually secondary disorder
membranous nephropathy disease
AutoAb against phospholipid receptor (PLA2-R) on podocyte of glomerulus
immune complex deposition in glomerular capillary walls and thickening of GBM
no hypercellularity on biopsy
membranous nephropathy medication induced
medications for another disease cause issue
malignancies: Lung and GI
infections: HBV, endocarditis, syphilis
autoimmune dz: SLE, thyroiditis, mixed connective tissue
drug exposure: NSAID
membranous nephropathy
typical symptoms and signs of nephrotic syndrome
AKI is rare
normal BP and normal GFR
membranous nephropathy diagnosis
serologic assays for PLA2R Abs
does not req. renal biopsy
disease conditions in membranous nephropathy
higher incidence of neoplasm - usual age appropriate screenings (Chest CT, colonoscopy, PSA, mammogram, pap/pelvic)
high risk of thromboembolic event
high risk of renal vein thrombosis
treatment of membranous nephropathy
risk stratification based on 24 hr urine protein level and Cr clearance
Low: ACE/ARB, BP/hyperlipidemia to goal
Moderate/HR: same +immunosuppression
excellent for renal transplant
treatment membranous nephropathy
low risk
proteinuria <3.5 g/day
low salt diet, strict BP control, ACE inhibitor
treatment membranous nephropathy
moderate risk
3.5-8 g/day normal renal fxn
low salt diet, strict BP control, ACE inhibitor +/- immunosuppression
treatment membranous nephropathy
high risk
8 g/day normal renal fxn
low salt diet, strict BP control, ACE inhibitor +immunosuppression
Focal Segmental Glomeurlosclerosis (FSGS)
primary idiopathic/ secondary to DM, GIV, vesicurethral reflux
nephrotic syndrome + evidence nephritic symptoms (microscopic hematuria at diagnosis, HTN)
FSGS renal biopsy
segmental areas of mesangial sclerosis
cloppase in some of glomeruli
primary FSGS presents + Tx
rapid decline of renal function
more likely to have nephrotic syndrome
Tx: high dose steroids for 2-3 months, taper
secondary FSGS presents + Tx
gradual decline in renal function over years
proteinuria may reach nephrotic levels, rarely develop nephrotic syndrome
supportive care + ACE inhibitors
tubulointerstitial disease
disease of kidney outside glomerulus
acute or chronic
acute tubulointerstitial disease (AIN)
hypersensitivity to medications
toxins/ischemia
interstitial edema, PMN infiltration, tubular cell necrosis
drugs that often cause AIN
ABX (PCN, Cephalosporins, sulfa)
NSAIDs
Rifampin
PPI
AIN patho
begins abruptly, AKI within days of exposure
subset of DRESS (drug rash with eosinophilia and systemic syndromes)
NSAID AIN
BEGINS WEEK TO MONTHS AFTER USE
mc in elderly
nephrotic syndrome
AIN presentation
rash fever eosinophilia eosinophiluria elevated IgE (allergic)
rifampin AIN
DOESN’T cause EOSINOPHILIA
usually occurs following reintroduction
DRSS
other causes of AIN besides drugs
immunologic disease
acute renal transplant rejection
infections (any type of infection will cause)
AIN treatment
promptly DC offending drug or treat underlying disease
document allergy
can hive high dose of steroid over 4-6 weeks
excellent supportive care
chronic tubulointerstitial disease
acute insult or progressive insult without acute cause
interstitial fibrosis and atrophy are often seen
main causes of chronic tubulointerstitial disease
obstructive uropathy
vesicoureteral reflux
analgesics
heavy metals
obstructive uropathy
prolonged obstruction of urinary tract (BPH, stone, carcinoma, retroperitoneal tumor/fibrosis)
UO alternates bt polyuria and oliguria
HTN + azotemia
back flow of uric into kidney
obstructive uropathy treatment
BPH and stones - usual tx
pelvic tumor: stent, tx underlying cause
retroperitoneal fibrosis
rare, encases ureters and abdominal organs in fibrotic tissue
can be caused by meds, biologic agents, cancers
diagnose by CT + contrast, CT biopsy
secondary = tx underlying dz + steroid
primary = steroid, tamoxifen, immunosuppressive
vesicoureteral reflux
urine passes retrograde during voiding due to incompetent vesicoureteral sphincter
disorder of child hood (typically renal damage before 5 y/o)
inflammatory response + fibrosis progressing to ESRD
VUR epidemiology
caucasian, red head, male neonates, female mc children
diagnosis of vesicoureteral reflux
voiding cystourethrogtaphy (VCUG)
recommended in children with UTI and siblings of persons with VUR
VUR treatment
keeping patient infection free
significantly decrease risk of renal damage (prophylactic ABX and monitored)
young patents - conservative tx,
analgesics chronic tubulointerstitial disease
ingestion of large quantities of analgesics for chronic pain (esp. combo pills)
MC in women
hematuria, mild proteinuria, polyuria, anemia, sterile pyuria
analgesics chronic tubulointerstitial disease patho
kidney tries to excrete large quantities
become concentrated at papillary tip (10x that of serum)
cause inflammation and papillary tip necrosis - sloughing
heavy metals that cause analgesics chronic tubulointerstitial disease
lead
cadmium
mercury
bismuth
accumulate and cause cell scaring, fibrosis
other causes of chronic tubulointerstitial disease
Lithium
imunologic dz
neoplasia
atherosclerotic kidney dz
genetics
metabolic d
general manifestations of chronic tubulointerstitial disease
polyuria due to inability to concentrate urine
may also have dehydration and salt wasting
hyperkalemia due to aldosterone restance
hyperCl due to renal tubular acidosis (decreased ammonia, inability to acidly, bicarb wasting)
diagnosis of chronic tubulointerstitial disease
UA non specific - brand waxy casts + proteinuria
ID underlying disorder
quantify degree of fibrosis on biopsy