Wilms tumor syndrome with absence of iris, genitourinary malformation, mental retardation/intellectual disability
WAGR (WT1)
Deny-drash
Wilms tumor, early onset nephrotic syndrom, male pseudohermaphroditism (WT1)
Beckwith-Wiedenmann
Wilms tumor, macroglossia, organomegaly, hemihypertorophy (WT2)
Wilms tumor genes and chromoso
WT1/WT2 on Chrom. 11
Eosinophilc epithelia tumor arising in collecting ducts
Renal oncocytoma (benign)
Pathogen of RCC
Loss of VHL on 3p. Increased IGF-1 and HIF transcription factor. HIF increased VEGF and PDGF
Drugs that cause AIN
Penicillins, cephalosporins, proton pump inhibitors, rifampin, cimetidine, sulfa diuretics
Medullary cystic disease
Inherited tubulointerstitial fibrosis and progressive renal failure. Can't concentrate urine. Medullary cysts usually not visualized. Shrunken kidneys on u/s.
Adverse loop diuretic effects
Ototoxicity, Hypokalemia, Dehydration, Allergy (sulfa), Acidosis, Nephritis (interstitial), Gout (OH DANG!)
Ethacrynic acid
Loop diuretic for those with sulfa allergy. More ototoxic!
Sodium cyanide test to check for cystine stones
1. Cyanide transforms cystine -> cysteine
2. Nitroprusside reacts w/ free sulfhydryl causing red discoloration.
Causes of fanconi syndrome
Cisplatin, tenofovir, expired tetracyclines, lead poisoning
Filtered load =
GFR x Px
Excretion rate =
V x Ux
Fractional excretion
Amount excreted/amount filtered
<1 = prerenal
>2 = renal or post-renal
FeNa
FENa = Pcr x UNa/ Ucr x Pna
Dopamine on kidney
Low dose: dilates interlobular arteries and arterioles to increase RBF but no change in GFR.
High dose acts as vasoconstrictor
Approximate the compensation in metabolic acidosis
1.5 [HCO3-] +8 +/- 2
Causes of Type 4 rta (low Aldo decreases PCT NH3 synthesis)
Low Aldo: ACE/ARB, NSAIDS, heparin, cyclosporine, adrenal insufficiency, Aldo resistance, k-sparing diuretics, obstruction nephropathy, TMP/SMX
Type 1 rta causes (alpha-intercalated defect)
Amphotericin B, analgesic nephropathy, congenital obstruction of urinary tract
Type 2 rta causes (PCT)
Fanconi syndrome and carbonic anhydrase inhibitors
Total Filtration Rate of Substance =
GFR x [plasma]
Net excretion rate =
Filtered rate - net tubular reabsorbtion
Net Reabsorption rate
Filtered rate - excretion rate
If positive there is net reabsorption.
If negative there is net excretion.
RPF = PAH clearance =
(U * V)/P
RBF =
PAH clearance/ (1-HCT)
Complication of maintenance stage ATN
Fluid overload, increasing CR and BUN, oligouria, hyperK+, high gap acidosis, high PO4 and Mg2
Complication of recovery stage of ATN
High volume diuresis causing electrolyte abnormalities. Decreased K, Mg, PO4, Ca, hypokalemia the most dangerous complication
PH = 6.1 + log [HCO3-] / (0.03 x PCO2)
HH equations