Pathoma - 12 (Kidney)

arold001's version from 2015-12-30 20:36

Section 1

Question Answer
What is Potters Sequence?BL renal agenesis --> oligoydraminios with lung hypoplasia, flat face with low set ears
Pt presents with UNILATERAL cystic kidney and cartilage tissuedysplastic kidney
what % risk of a child getting dysplastic kidney?0% !! its noninherited!
noninherited congenital malfomation of renal parenchyma characterized by cysts and ABNORMAL CARTILAGEdysplastic kidney (noninherited and unilateral)
baby presents with cystic kidneys and portal HTNARPKD
baby presents with cysts in the kidney AND liverARPKD
name 3 things associated with ADPKD?berry anuerysm, hepatic cysts, MITRAL VALVE PROLAPSE
what cardiac defect is associated with ADPKD?mitral valve prolapse
shrunken cystic kidneys are seen in what disease?medullary cystic kidney disease

Section 2

Question Answer
what causes prerenal azotemia?decreased blood flow to the kidneys --> activates RAA --> aldosterone causes reabsorption of Na+, water, and BUN --> increase BUN:Cr ratio
what causes postrenal azotremia?obstruction of urinary tract/ureters
brown granular casts in the urineATN
describe BUN, FENa, and urine concentrating ability in ATNdecreased reabsorption of BUN (BUN:Cr<15), decreased reabsorption of Na+ (FENa >2%) and inability to concentrate urine (urine osmolarity <500)
what are the 2 main causes of acute tubular necrosis?ischemia and nephrotoxicity
what 2 areas of the nephron are most susceptible to ischemia?proximal tubule and medullary segment of tAL
what are some nephrotoxic causes of ATN?aminoglycosides, lead, myoglobinuria (from muscle crush injury), ETHYLENE GLYCOL, radiocontrast dye and urate (tumor lysis syndrome)
what drug is given prior to chemotherapy to prevent urate-induced ATN?allopurinol and hydration
what is acute interstitial nephritis?DRUG-INDUCED HYPERSENSITIVITY involving interstitium and tubules
name 3 drugs that cause AINNSAIDS, penicillins, diruretics
eosinophils in the urine is characteristic of what condition?acute interstitial nephritis (AIN)
sickle cell disease, phenacetin and aspirin use can cause which renal disease?renal papillary necrosis

Section 3

Question Answer
what are the 6 nephrotic syndromes?1) minimal change disease
3) membranous nephropathy
4) membranoproliferative glomerulonephritis
5) diabetes
6) amyloidosis
describe nephrotic syndrome?proteinuria >3.5g/day, hypoalbuminuria (pitting edema), hypogammaglobulinemia (increased risk of infection), hypercoagulable state (loss of antithrombin III), hyperlipidemia and hypercholesterolemia
what cancer is associated with minimal change disease?Hodgkin lymphoma = get massive production of cytokines from Reed Sternberg cells that damage foot proceses
what causes flattening of the foot processes in minimal change disease?cytokine production that injures/flattens foot processes
whats the tx for MCD?steroids = excellent response! damage is mediated by cytokines from T cells
whats the most common cause of nephrotic syndrome in Hispanics and blacks?FSGN
whats the most common cause of nephrotic syndrome in HIV or heroin abusers?FSGN
whats the most common cause of nephrotic syndrome in pts with sickle cell disease?FSGN
pts with HIV develop nephrotic syndrome....which subtype?FSGN
what does "membranous" mean?thickening of the BM due to immune-complex deposition
whats the most common cause of nephrotic syndrome in white pts?membranous nephropathy
which nephrotic syndrome is assocaited with hep B or C?membranous nephropathy and Type I membranoproliferative glomerulonephritis
nephrotic syndrome that shows subEPITHELIAL deposits with "spike and dome" appearance on EMmembranous nephropathy
why does membranous nephropathy show subepithelial deposits with spike and dome appearance?subepithelial cells (aka foot processes) like to only sit on the BM, and hence response to subepithelial deposits by thickening BM
thick glomerular BM with "tram track" appearancemembranoproliferative glomerulonephritis
whats the role of C3 nephritic factor?autoantibody that stabilizes C3 convertase so it cant be degraded (C3 --> C3a + C3b), leading to overactivation of complement, inflammation and low levels of circulating C3
subendothelial depositsType I MPGN
dense depositsType II MPGN
nephrotic syndrome a/w C3 nephritic factorType II MPGN
whats the tx of diabetic nephrotic syndrome?ACE Inhibitors

Section 4

Question Answer
describe nephritic syndromeglomerular inflammation and bleeding, limited proteinuria (<3.5g/day), oliguria, azotemia, periorbital edema and HTN, RBC casts and RBC in urine
biopsy shows hypercellular inflammed glomerulinephritic syndrome = glomerular inflammation and bleeding
what causes damage in nephritic syndromes?immune-complex deposition activates complement --> C5a attracts neutrophils which cause DAMAGE!
nephritogenic strains of GAS contain what protein?M-protein virulence factor
nephritic syndrome that shows subepithelial humps on EMpost-streptococcal glomerulonephritis
what are the crescents in RPGN made out of?fibrin and macrophages = inflammatory debri
pt presents with hematuria and hemoptysis...diagnosisgoodpasture syndrome
whats the most common type of renal disease in SLE?diffuse proliferative glomerulonephritis or membranous nephropathy
what 3 renal disease show negative IF (pauci-immune)?wegener granulomatosis
microscopic polyangitis
churg straus
granulomatous inflammation, eosinophilia, asthma and renal diseasechurg strauss syndrome
another name for Berger diseaseIgA nephropathy
whats the most common nephropathy WORLDWIDE?IgA nephropathy = usually follows mucosal infections (gastroenteritis)
what defect is seen in alport syndrome?defect in Type IV collagen
pt presents with hematuria, sensory hearing loss and vision changesAlport syndrome = hematuria, hearing loss, eye problems

Section 5

Question Answer
what causes positive leukocyte esterase in urine dipstick?pyuria
if urine dipstick shows positive nitrites, what does taht mean?bacteria converts nitrates to nitrites
what is sterile pyuria?presence of pyuria (>10 WBC/hpf and leukocyte esterase) with NEGATIVE URINE CULTURE
pyuria with NEGATIVE urine culture...think of what?urethritis due to Chlamydia trachomatis or Neisseria gonorrhea
WBC castacute pyelonephritis
what 3 bacterias cause acute pyelonephritis?E. coli > klebsiella > enterococcus faecalis
what renal disease causes thryoidization of the kidney?chronic pyelonephritis = atrophic tubules containing eosinophilic proteinaceous material resembling thyroid follicle

Section 6

Question Answer
what type of kidney stones is a/w Crohns disease?calcium oxalate
whats the tx for calcium oxalate kidney stones?thiazides
what 2 bacterias cause ammonium magnesium phosphate kidney stones?proteus vulgaris or klebsiella = urease positive orgs
which kidney stone is radiolucent/NOT seen xray?uric acid kidney stones
what causes cysteine kidney stones in kids?cystinuria = defect of tubules that results in decreased reabsorption of cysteine
staghorn calculi in adults? kids?adults: AMP stone. kids: cysteine stone

Section 7

Question Answer
what cells produce Epo?renal peritubular interstitial cells
what causes hypocalcemia in chronic renal failure?decreased 1-alpha hydroxylation of vit D by proximal renal tubule cells
end stage renal failure has an increased risk for which carcinoma?renal cell carcinoma
how does the kidney look in end stage renal disease?shrunken kidneys with cysts
angiomyolipoma is associated with what disorder?tuberous sclerosis
pt presents with hematuria, palpable mass and flank painrenal cell carcinoma
renal cell carcinoma is a paraneoplastic syndrome that can secrete what?Epo, renin, ACTH, PTHrP
Explain why renal cell carcinoma can cause left sided varicocelerenal cell carcinoma loves to invade/spread to LT renal vein --> blocks drainage of LT spermatic vein
Explain the pathogenesis of renal cell carcinoma (hint - has to do with VHL gene)loss of VHL tumor suppressor gene --> leads to increased IGF-1 (promotes growth) and increased HIF transcription factor (increases VEGF and PDGF) --> promtoes angiogenesis and tumor growth
yellow mass seen on kidney?renal cell carcinoma
Whats the hallmark cell of wilms tumorblastema = immature kidney mesenchyme

Section 8

Question Answer
Whats the most common malignant renal tumor in kids?wilms tumor
Elderly pt presents with PAINLESS hematuriaurothelial (transitional cell) carcinoma
What are the 2 pathways of developing urothelial transition cell carcinoma?1) flat (early p53 mutations) = high grade flat tumor then invades
2) papillary (NOT early p53 mutation) = low grade papillary tumor --> high grade papillary tumor and then invades
What are some risk factors for bladder squamous cell carcinoma?chronic cystitis, schistosoma hematobium, long-standing kidney stone
What does bladder adenocarcinoma arise from?urachal remnant (urachal duct = connects fetal bladder with yolk sac --> lined by glandular cells that are capable of adenocarcinoma)