Chapter 12 - Kidney and Urinary Tract
Terms in this set (93)
What is the most common congenital renal anomaly?
What does the horseshoe kidney get stuck on during its ascent into the abdomen?
Root of the inferior mesenteric artery
What is Potter's sequence? What is is caused by?
Lung hypoplasia, low set ears and flat face, abnormalities of extremities
What are the demographic differences in AR vs. AD PKD?
AR - presents in infancy
AD - young adulthood
A baby with congenital hepatic fibrosis leading to portal HTN, think
How does the AD form of PKD present? What are the associated manifestations?
HTN due to increased renin, hematuria, reanl failure
Associated with MVP, berry aneurysm, hepatic cysts
What is the mutated gene in AD PKD?
Where are the cysts in medullary cystic kidney disease vs. PKD?
Medullary - collecting ducts
PKD - cortex and medulla
What is the inheritance of medullary cystic kidney disease?
How would you distinguish medullary cystic kidney disease from other cystic kidney diseases?
Medullary presents with fibrotic, shrunken kidneys (the others will be enlarged)
What is azotemia?
Inc BUN and creatinine
What will the BUN:Cr ratio be in prerenal azotemia?
BUN:Cr will be >15. BUN can be reabsorbed but Cr cannot, so in the attempt to reabsorb water/H20, BUN will also be reabsorbed
[FENa]<1%, urine osmolarity >500 - indicates that the tubules are still functional
What will the BUN:Cr ratio be in postrenal azotemia (early)?
>15 (due to back pressure forcing BUN into the blood)
FENa <1%, urine osm > 500
What will the BUN:Cr ratio be in ATN (intrarenal azotemia)?
Tubules are no longer reabsorbing and concentrating due to damage
What kind of casts do you see in ATN?
Why does the GFR decrease in ATN?
Necrotic cells plug tubules and increase the back pressure
What are the portions of the nephron most susceptible to ischemic injury?
Proximal tubule and medullary segment of the thick ascending limb
What are some toxic agents that can cause ATN?
Myoglobinuria (crush injury to muscles)
Urate (from tumor lysis)
Chemotherapy causes massive destruction of tumor (and other) cells, releasing lots of nuclear material and thus urate, which is harmful to the kidney. What can we give prior to chemo to combat this?
Hydration and allopurinol
What is associated with oxalate crystals in urine?
Ethylene glycol ingestion
What is the acid/base disorder in ATN?
Acidosis - can't excrete organic acids
What is the K+ level in ATN?
Hyperkalemia (can't excrete)
What are some causes of acute interstitial nephritis?
What is pathognomonic for acute interstitial nephritis?
Pt presents with oliguria, fever, and rash a few days after being started on an NSAID. Think:
Acute interstitial nephritis
What are some causes of renal papillary necrosis?
Chronic analgesic abuse (phenacetin, aspirin)
Severe acute pyelonephritis
Nephrotic syndrome is characterized by proteinuria >__/day. What kind of casts do you see?
Fatty casts (the liver is putting out more lipids into blood to help with the oncotic pressure lost from proteinuria)
What is the main anticoagulant protein lost in nephrotic syndrome?
What nephrotic syndrome may be seen in pts with Hodgkin lymphoma?
Minimal change disease
This nephrotic syndrome will have negative IF, a normal glomerulus, effacement of foot processes, and selective proteinuria (albumin, not Ig)
Minimal change disease
This nephrotic syndrome is associated with HIV, heroin use, and sickle cell disease
What is the most common nephrotic syndrome in Hispanics and AAs?
What is the most common nephrotic syndrome in Caucasians?
If a minimal change disease doesn't respond to steroids, it may be
The most common nephritic syndrome in SLE is __. The most common nephrotic syndrome is __
Diffuse proliferative GN
What things are associated with membranous nephropathy?
Drugs - NSAIDs, penicillamine
Where are the deposits in membranous nephropathy? Membranoproliferative?
Subepithelial (right under the podocytes)
Subendothelial (Type I) and intramembranous (Type II - in the BM)
What nephrotic syndrome is associated with "spike and dome" appearance on EM?
What are the two types of membranoproliferative GN and what are they associated with?
Type I - subendothelial - Hep B/C
Type II - intramembranous - C3 nephritic factor (an autoantibody that stabilizes C3 convertase increasing complement activation)
Which nephrotic syndrome has a "tram-track" appearance on EM?
Pathology of DM induced nephrotic syndrome?
Hyaline arteriosclerosis from nonenzymatic glycosylation of BM leads to narrowing of the efferent arteriole - inc back pressure and hyperfiltration.
Sclerosis of mesangium and Kimmelstiel-Wilson nodules
What can you use to slow the damage from DM nephrotic syndrome?
What kind of casts do you see in nephritic syndrome?
Post-streptococcal GN arises after infection with __ __ strep. The nephritogenic strains carry the __ virulence factor.
Group A, B-hemolytic
What is the classic microscopic finding in RPGN?
Crescents in Bowman's space (made of fibrin and macrophages)
In Berger disease, where does the IgA deposit?
When does Berger disease present?
What is Alport syndrome? What is the inheritance?
Defect in Type IV collagen causing BM thinning/splitting. Hematuria, sensory hearing loss, and ocular disturbances.
Lab findings that would indicate cystitis:
+Leukocyte esterase and nitrites
What presents as subepithelial "humps
What is the IF pattern in Goodpasture's syndrome? What is the Ab?
Linear; anti-collagen/BM Ab
What demographic do you see Goodpastures syndrome in?
Young adult males
Which diseases are pauci-immune on IF?
How do you distinguish between Wegener's, microscopic polyangiitis, and Churg-Strauss syndromes?
Wegener's - C-ANCA
C-S and microscopic - P-ANCA
C-S also has eosinophilia, granulomatous inflammation, and asthma (microscopic does not)
Which RPGN will present first with nasopharyngeal Sx, Wegener's or Goodpasture's?
What does a sterile pyuria with dysuria suggest?
Urethritis due to chlamydia or gonorrhea infection
When do you see WBC casts in urine?
When infection has ascended to pyelonephritis
What are the most common causative organisms in pyelonephritis?
What is "thyroidization" of the kidney? What is it seen in?
Atrophic tubules full of proteinaceous material, look like the thyroid. Seen in chronic pyelonephritis.
Where do you see waxy casts?
What are the clinical features of chronic renal failure?
Encephalopathy with asterixis
Salt and water retention (HTN)
Anemia (dec EPO)
Hypocalcemia (dec 1-alpha-hydroxylase)
Why does uremia cause bleeding disorders?
Uremia inhibits platelet aggregation
Buildup of nitrogenous waste products
What cells of the kidney produce EPO? What cells produce 1-a-hydroxylase?
Peritubular interstitial cells
Proximal renal tubule cells
What is renal osteodystrophy?
Due to 2ndary hyperPTH - increased resorbtion of bone causes damage
Osteomalacia - osteoblasts lay down osteoid that cannot be mineralized
Angiomyolipomas in the kidney, think
RCC arises from
What is the classic triad of RCC?
How do you treat Ca stones?
Hydrochlorothiazide - Ca sparing diuretic, dec Ca in urine
What kind of stones are seen in Crohn's disease?
What is the most common type of nephrolithiasis?
Ammonium magesium phosphate stones are most commonly due to
Infection by urease + organisms (Klebsiella, proteus) leading to alkaline urine
Uric acid stones are __(radiolucent/radiopaque)
What are some etiologies of uric acid stones?
Low urine volume (hot, arid climates)
Hyperuricemia (leukemia or myeloproliferative disorder)
How do you treat uric acid stones?
Hydration, alkalinization of urine (K, bicarb)
Cysteine stones are most commonly seen in what demographic? How do they form?
Cysteinuria (genetic defect of tubules leading to dec reabsorption of cysteine)
Forms staghorn calculi
How do you treat cysteine stones?
Hydration and alkalinization of urine
A L sided RCC could present with this unique clue, due to anatomy
L sided varicocele due to the L spermatic vein draining into the L renal vein
What are some hormones causing paraneoplastic syndromes that you could see in RCC?
RCC most commonly presents as this type microscopically
Pathogenesis of RCC
Loss of VHL (3p) - a tumor suppressor
Leads to inc IGF-1 and HIF transcription factor (increases VEGF and PDGF)
Sporadic vs. hereditary renal tumors - demographics, pathogenesis, location
Sporadic - adult males, smoking, upper poles
Hereditary - younger adults, Von-Hippel-Lindau disease (AD, inactivation of VHL gene), bilateral and multiple
Von-Hippel-Lindau disease is associated with these two types of malignancies
Hemagioblastoma of the cerebellum
Where does renal cancer spread?
Lung and bone
Wilms tumor demographics and defn.
Children 3 years
Malignant tumor composed of blastema (immature kidney tissue)
How does a Wilm's tumor present?
Large unilateral flank mass with hematuria and HTN
What is the mutation in Wilms tumor?
Aniridia (absence of iris)
Retardation (mental and motor)
Risk factors for urothelial (transitional cell) ca
Long term cyclophasphamide or phenacetin
Flat vs papillary pathway of urothelial ca
Flat begins as high grade then invades, associated with early p53 mutation
Papillary begins as low grade, then progresses to high grade and then invasion, not associated with p53
What is meant by a "field defect" in a urothelial ca?
Since the entire urothelium has been hit, most/all of the cells are mutated and at risk. Tumors are multifocal and recurring
Risk factors for squamous cell ca of bladder
Needs squamous metaplasia first: chronic cystitis, schistosoma hematobium infection, long standing nephrolithiasis
What is the classic demographic for squamous cell ca of bladder?
How do you get adenocarcinoma of the bladder?
Cystitis glandularis (columnar glandular metaplasia due to chronic cystitis)
Existrophy (failure to form caudal portion of anterior abdomen and bladder walls - exposes bladder to the outside)
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