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

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