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Buzz Words- Renal

Buzz words and hallmark presentations (path, thomas, zel, ryan- tubulo ds)

questionAnswer
complement levels in IgA nephropathy? NORMAL C3 and C4 key to DDx from Post-infectious GN in which complement levels are LOW (also IgA occurs <b>days<b/> post infection; Post Infectious GN occurs weeks later)
oval fat bodies may be sign of? associated with what type of syndrome? hyperlipidemia; nephrotic syndrome
classic presentation: proteinuria (foamy urine), hypoalbuminemia, edema, hyperlipidemia nephrotic syndrome
likely to present in children with acute onset; associated with lymphomas*; caused by effacement of podocytes minimal change disease
most common cause of nephrotic syndrome in african americans? FSGS
nephrotic syndrome most commonly associated with HIV/AIDS FSGS
which nephrotic syndrome is an exception in that it is due to an antibody/antigen complex? membranous glomerulonephropathy
which syndrome is it crucial to perform age appropriate cancer screening? membranous GN
kimmel steil- wilson nodules diabetic glomerulosclerosis or as Bri says... Jimmy Kimmel has diabetes (but he doesn't?)
classic presentation: hematuria, oliguria, HTN (+ RBC casts and low protein) nephritic syndrome
crescentic lesions (general) nephritic syndrome
#1 cause of GN worldwide IgA nephropathy
associated with Henoch Schonlein Purpura IgA nephropathy
IgA nephropathy is associated to occur after which two broad kind of infections? URI and GI infections
Post-infectious is associated to occur after what kind of infection usually? Strep Pharyngitis
which nephritic syndrome presents with low C3 and low C4? Post infectious GN
which nephritic syndrome presents with low C3 and normal C4? Membranoproliferative GN
which nephritic syndrome presents with normal C3 and normal C4? IgA nephropathy
dry mouth, dry eyes; positive anti-ro and anti-la antibodies Sjogrens disease (commonly associated with membranoproliferative GN)
Anti-GBM only kidney involvement- more likely in? older women
Anti-GBM- goodpasture (with kidney and lung involvement)-- more likely in? young men with history of smoking (may present with hemoptysis
nephritic syndrome with lung, kidney AND SINUS involvement Wegener's granulomatosis (a type of small vessel vasculitis); sinus involvement may include- epistaxis or recurrent sinusitis must Ddx from Goodpastures (kidney and lung only)
c-ANCA (+) AND anti-PR3 Wegener's
hansel stain (eosinophilia) acute interstitial nephritis
sterile pyuria and HTN chronic interstitial nephritis
brown muddy casts acute tubular necrosis
tubulointerstitial disease associated with fanconi's syndrome renal tubular acidosis (type II)
acquired fanconi's syndrome multiple myeloma
tamm-horsfall mucoprotein cast nephropathy assoc. with multiple myeloma
congo red stain with beta pleats amyloid assoc. with multiple myeloma
Benz-jones proteins multiple myeloma
onion skin lesions (hyperplastic arteriolitis) malignant HTN
fibrotic intimal thickening (luminal narrowing) HTN nephrosclerosis (benign)
high levels of renin in elevated renal vein renal artery stenosis
string of beads on angiogram fibromuscular dysplasia
schistocyes in peripheral blood smear microangiopathic thrombotic disease (also seen in pentad of symptoms with TTP)
classic presentation: pentad-- fever, neurologic symptoms, microangio hem. anemia, thrombocytopenia, ARF TTP
ADAMTS13 and vWF TTP
WAGR syndrome + WT1/WT2 mutations nephroblastoma (wilm's)
most common renal tumor of infancy congential mesoblastic nephroma
most common renal tubular epithelium neoplasm renal papillary adenoma
mahogany brown; stellate central scar renal oncocytomas
LM: mix of adipose, smooth muscle, blood vessels angiomyolipoma (duh look at the word.. angio-- vessel, myo-- muscle, lipo--fat)
VHL gene mutation leading to high levels of VEGF RCC (more specfically in clear cell) mutation occurs in over 90%!!!
triad of sym: flank pain, palpable mass, hematuria RCC
most common RCC clear cell- conventional
C-met gene Papillary RCC (also assoc. with RF/ "dialysis kidney")
renal tumor assoc. with sickle cell (african am and mediterranean) renal medullary carcinoma very rare, very aggressive
urothelial invasion of the bladder collecting duct carcinoma (worst prognosis of typical RCC subtypes)
known to invade the renal vein and IVC RCC
dilation of renal pelvis and calyces & atrophy obstructive uropathy
neoplasm associated with urachal cysts adenocarcinoma
soft tan broad mucosal plaques malacoplakia
foamy macrophage, multinucleated cells with granular PAS positive malacoplakia (note: amyloid is PAS negative)
michaelis gutmann bodies malacoplakia
developmental anomoly associated with Trisomy 18 horseshoe kidney
oligohydraminos and Potter's sequence (fatal condition) bilateral renal agenesis **TOL said to know** (potters- flat nose, low set ears, recessed chin)
cobblestone gross appearance kidney ADPKD
development path associated with berry aneurysms ADPKD
develpment path associated with hepatic fibrosis ARPKD
clinical triad: abnormal abdominal musculature, UT anomalies, bilateral cryptorchidism prune belly syndrome
cobra head appearance on IVU ureterocele
hallmark of costovertebral angle tenderness ureteral calcuil
WBC casts in UA pyelonephritis
Balanitis is commonly associated with? Phimosis
hallmark of perineal discomfort and fever bacterial prostatitis
bladder pain relieved by voiding interstitial cystitis
glomerulations-- submucosal hemorrhage interstitial cystitis
christmass tree bladder bladder diverticuli (neurogenic)
tender, enlarged epididymis AND assoc with arthritis epididymitis
clinical triad: mass, hematuria, pain RCC (kidney)
requires Lasix for Dx UPJ obstruction
one exception of nephrotic diseases in which renal biopsy is NOT indicated MCD in children
No finding on LM and IF; podocyte effacement on EM Minimal change disease note: FSGS may also show similar findings if focal segment is missed on biopsy (IF will also show collapse of capillaries and increase in ECM)
the only nephrotic with immune complex deposits membranous glomerulopathy (this is a repeat but was mentioned with both thomas and leonard)
LM: thick capillary walls with silver stain spikes IF: granular stainging EM: subepithelial immune deposits* membranous glomerulopathy (remember only nephrotic with immune complexes)
FSGS must be DDx with? MCD
leading cause of ESRD; often presents with small vessel disease and microalbumin Diabetic GS
LM: thickening of BM region AND proliferation/expansion of mesangium Diabetic GS (DDx with FSGS- this occurs only in the glomerular capillary)
hallmark of back pain with lytic lesions on xray multiple myeloma (associated with Amyloid nephropathy)
congo red stain and apple green bifringement with polarized light amyloid nephropathy (remember it is PAS negative! unlike Diabetic GS)
LM: proliferative GN IF: "lumpy-bumpy" deposits EM: subepithelial "humps" Acute post infectious GN
EM: immune complex deposits subendothelium and mesangial hypercellularity Type I MPGN
"tram tracking" of GBM on silverstain Type I MPGN
deposit of complement and ribbon like increase in GBM density Type II MPGN
associated with C3 nephritic factor (IgG autoAb) Type II MPGN
common presentation in female african americans with SLE Lupus nephritis (think malar rash with nephritic symptoms)
Morphologic: thick capillary loops--"wire loops" Lupus nephritis
ANA (+) with anti-dsDNA antibodies Lupus nephritis Dx
associated with autoantibody against IV collagen anti-GBM GN
IF: smooth diffuse "linear" IgG staining Anti-GBM GN (as opposed to "lumpy" with acute post infectious and "granular" with membranous glomerulopathy)
prominent hyaline sclerosis in renal arterioles diabetic GS (FSGS also shows sclerotic changes but only in the GC not the arterioles)
most common cause of nephrotic syndrome in kiddos minimal change disease
most common cause of nephrotic syndrome in US caucasian/asian adults? african american? membranous glomerulopathy; FSGS
glomerular disease with favorable prognosis with corticosteroid Tx Minimal change disease
classic triphasic histopath of nephroblasomta blastema (primitive tubules), epthielium (abortive tubules), stroma (spindle cells)
three syndromes assoc with nephroblasomta beckwith-Wiedeman, *WAGR syndrome*, denys drash WAGR- genital anomaly retardation
tumor characterized by spindled cells resembling immature tissue; metastases rare congential mesoblastic nephroma (Ddx: wilms tumor-- commonly metastases to lung)
most common renal tubular epithelium neoplasm renal papillary adenoma
renal papillary adenoma commonly associated with what? acquired cystic renal disease
rank in order of worst prognosis to best clear cell RCC, Papillary RCC, Chomophobe RCC, collecting duct RCC collecting duct (worst)-->clear cell--> papillary-->Chomophobe (Best)
carcinoma associated with "carcinogenic field effect" and presence of mulifocal neoplasms renal urothelial carcinomas
commonly associated with hepatic fibrosis and portal hypertension ARPKD
primary genetic abnormality PKD1 or PKD2; what do these encode? ADPKD; encode polycystin
primary genetic abnormality PKHD1; what does this encode? ARPKD; encodes fibrocystin (think ARPKD-- hepatic FIBROSIS (hard liver=H in PK*H*D1)
acquired condition which may be hemorrhagic and confused with cystic RCC** simple renal cyst*** must do microscopic evaluation to Dx -- will see thin walled uniloculated in simple renal cysts
degenerative kidney, bumpy shrunken gross appearance leading to ESRD and dialysis acquired renal cystic disease
Abx induced acute interstitial nephritis clinical triad: fever, rash, eosinophilia
high urine glucose and normal serum glucose chronic interstitial nephritis
characterized by clinical presentation of acidosis and hyperkalemia from prolonged hypotension (septic shock) acute tubular necrosis (think brown muddy casts)
renal tubular acidosis with hyperkalemia, hyperchloremic met. acidosis type 4 - high serum K (distal)
renal tubular acidosis with hypokalemia and acidosis type 1- low serum k often presents in kids as failure to thrive and renal stones
low back pain, male age>50, african american hepatosplenomegaly, cardiomyopathy multiple myeloma
most common cause of Multiple myeloma cast nephropathy (*tamm-horsfall mucoprotein)
DDX between RTA and Multiple myeloma Anion gap RTA (normal AG) Multiple myeloma (low AG)
which disease is assoc with acquired fanconis ? which is caused by fancois synd? acquired-- multiple myeloma caused by fanconis syndrome-- type II renal tubular acidosis (also pharm plug-- tetracyclines that have expired are linked with acquired fanconis)
potassium disorder; hypertensive, low renin, low aldosterone Liddles-- hypokalemic
K disorder; normotensive, HIGH urine Ca Bartters-- hypokalmeic
K disorder; normotensive, NORMAL urine Ca and low serum Mg Gitelmans-- hypokalemic
K disorder; HTN, low renin activity hyperK withOUT renal failure Gordans (pseduohyopaldosteronism) aldosterone resistant-- impaired renal excretion
EKG: ST depression, decreased amplitude, U WAVE* hypokalemia
EKG: Tinted T waves, no P wave, wide QRS hyperkalemia
if urine Osm > serum Osm Dx likely? SIADH
what is the lethal dose of ASA? 500 mg/dl
toxic dose of ASA? 150 mg/kg or >40 mg/dL
3 Tx options of ASA OD charcoal, sodium bicarb, hemodiaylsis (if neurologic symptoms)
acid base disturbances seen with ASA OD resp alkalosis and metabolic acidosis (SEPERATE and INDEPENDENT)
SSRIs (sertraline, fluoxitine, citalopram) lead to what common disorder in older people? hyponatremia
most common cause of staghorn calculi UPJ obstruction
Created by: caldwekl
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