2/18/99

Pathology II

 

Diseases Affecting tubules

1.        inflammatory involvement of tubules and interstitium

2.        ischemic or toxic tubular injury

 

Pyelonephritis—bacterial infections in renal pelvis

Interstitial nephritis—non infectious causes

 

Acute pyelonephritis

·         common suppurative inflammation by bacterial infection

·         enteric gram negative rods—

·         E. coli—most common

·         Proteus, Klebsiella, Enterobacter, Pseudomonas—in recurrent infections

 

2 Routes

·         Hematognous—less common

·         Ascending infection—lower urinary tract—most important route

·         Colonization of distal urethra and female introitus

·         Movement into bladder against urine flow

·         Urethral instrumentation:  catheterization/cysoscopy

·         Female more prone due to short urethra and urethral trauma during sexual intercourse

 

 

Bladder Defenses

1.        antimicrobial properties of bladder mucosa

2.        flushing action w/voiding of urine

 

Predisposing factors

·         obstruction-urinary stasis-leads to bacterial proliferation and ascent to renal pelvis

·         more common in patents w/urinary tract incompetence of vesicoureteral orifice

·         normal-1 way valve preventing retrograde urine

·         flow-incompetent valve allows reflux of urine to bladder

·         diabetes

·         septicemia

·         necrotizing papillitis

 

Morphology

·         suppurative necrosis or abscess formation w/I renal substance

·         early stages:  limited to interstitial tissue

·         later stages:  abscess rupture into tubules

·         white cell casts:  in urine from neutrophils in collecting ducts—Pyelonephritis is the most common disease associated w/ WBC casts

·         note:  casts form when proteins precipitate and get in the tubular lumina, it is generally held that Tamm-Horsfall mucoproteins from the basic matrix of the all casts.  WBC’s enter tubular lumina from the interstitium

 

Pyelonephrosis-due to obstruction blocking the drainage of suppurative exudate

Necrotizing papillitis or Papillary necrosis-complication more common in diabetes

·         pahognomonic feature is sharply defined gray-white to yellow necrosis of the apical 2/3

 

clinical course

·         sudden onset-pain at costovertebral angle

·         systemic signs of infection-chills, fever, malaise

·         puruia, bacteriuria

·         bladder irritation-dysuria, frequency, urgency

·         course tends to be self-limiting

·         recurrent or chronic if predisposing factors are present

·         poor prognosis w/ necrotizing papillitis-sepsis and renal failure

 

Chronic Pyelonephritis and Reflux Nephropathy

·         important cause of chronic renal failure

·         interstitial inflammation and scarring of the renal parenchyma w/grossly visible scarring and deformity of the pelvicalyceal system

 

1.        Chronic Obstructive Pyelonephritis

·         Recurrent infections due to persistent obstructive lesions-posterior urethral valves, calculi, or obstructive anomalies of the ureter

 

2.        Chronic Reflux Pyelonephritis

·         More common forms, recurrent infections and  damage due to persistent reflux

 

Clinical Course

·         Present late

·         Don’t know what is going on—gradual

·         Pyelograms show asymmetrically contracted kidney w/ blunting and deformity of the calyceal system

·         Glomerular lesions ultimately develop, eventually leading to renal failure

 

Interstitial Nephritis

·         Due to use of ab and analgesics

·         Acute drug-Induced interstitial nephritis

·         Adverse reaction-synthetic penicillin's, methicillin, ampicillin, rifampin, diuretics (thiazides, NSAIDS

 

Clinically

·         15 days (range 2-4days) after exposure

·         fever, eosinophilia, skin rash

·         renal abnormalities, hematuria, mild proteinuria, leukocyturia

·         acute renal failure w/ oliguria (esp. in older patients)

 

Morphology

·         interstitial edema w/ lymphocytes and macrophage infiltration

 

Pathogenesis

·         immune mechanism is probable

·         hypersensitivity suggested by latent period, eosinaphilia, skin rash

 

Analgesic Nephropathy

·         patients consume large quantities of analgesics may develop chronic interstitial nephritis, often associated w/renal papillary necrosis

·         mixtures of drugs is more common:  phenacetin, aspirin, acetaminophen, caffeine, or codeine for long periods

 

Clinical Features

·         chronic renal failure

·         hypertension

·         anemia

 

Acute Tubular Necrosis (ATN)

·         acute tubular necrosis is a clinicopathlogic entity characterized morphologically by destruction  of tubular epithelial cells and clinically by acute suppression of renal fxn

·         most common cause of acute renal failure

 

Clinical Patterns

1.        ischemic ATN-inadequate blood flow, hypotension, shock, severe trauma, hemolytic crises, mismatched blood transfusions

2.        Nephrotoxic ATN-caused b poisons, heavy metals, e.g. mercury, organic solvents, e.g. carbon tetrachloride, radiographic contrast media

 

Pathogenesis

·         Tubular injury-damage to epithelial cells, debris blocking urine outflow

·         Increase in intratubular pressure causes decrease GFR

·         Blood flow distrubances-intrarenal vasoconstriction causing decrease GFR and decrease O2

 

Clinical

·         Initiating phase

·         Maintenance phase—2-6days. Urine output falls dramatically.  Signs of symptoms of fluid overload

·         Recovery-steady increase in urine vol., increased vulnerability to infection

 

Cystic Diseases of the Kidney

·         Simple Cysts

·         Cortical, 1-5 cm, common post mortem finding

·         Differentiate from tumor

 

Autosomal Dominant (Adult) Polycystic Kidney Disease

·         Multiple expanding cysts of both kidneys that destroy parenchyma

·         Incidence:  1 in 1000

·         10% of cases of chronic renal failure

 

Clinical

·         no symptoms until 40 y/o

·         flank pain-heavy dragging sensation

·         excruciating pain w/acute distention of cyst

·         intermittent gross hematuia and urinary infection

·         hypertension in 75% of patients

·         saccular (berry)aneurysms-Circle of Willis-15%-30%

·         asymptomatic liver cysts in 1/3rd

·         ultimately fatal-end stage renal failure about age 50

·         renal transplantation

 

Urinary Outflow obstruction

Renal Stones

·         75% calcium oxalate or calcium oxalate/calcium phosphate

·         15%-magnesium ammonium phosphate

·         10%-uric acid or cystine

·         all stones have an organic matrix of mucoprotein

·         the most important cause is increased urine concentration of the stone’s constituents, so that it exceeds their solubility in urine (supersaturtion)

 

Calcium Stones (Table 14-4)

·         50% of patients have hypercalciuria w/no hypercalcimia

·         absorb excessive amounts of calcium form the gut and excrete into the urine.  Some have a primary renal defect of calcium reabsorption

·         5-10% have hypercalcemia w/hypercalciuria (hyperparathyroidism, vit D intoxication , sarcoidosis)

·         20% have excessive excretion of uric acid in urine-thought to provide a nidus for calcium deposition

 

Magnesium ammonium phosphate (struvite) stones

·         Alkaline urine-due to urinary tract infections

·         Proteus vulgaris and staph are urea-splitting bacteria and predispose to stones

·         Bacteria can sever as nidi for any kind of stone

·         Avitaminosis A – desquamated squamous from metaplastic epithelium of collecting system

 

Uric Acid stones (acidic urine)

·         gout and diseases involving rapid cell turnover, such as leukemia

·         high uric acid levels (hyperuricemia, increased urine urate) and development of stones

·         50% of patients w/ uric acid stones have neither, but a tendency for acid urine (pH<5.5)

 

Cystine stones (acidic urine)

·         genetically determined defect in renal transport of cystine

 

Morphology

·         unilateral in 80% of patients

·         common sites-renal pelvis, calyces, bladder

·         tend to be small (2-3mm)—smooth or jagged

·         Staghorn calculi—cast of renal pelvis and calyceal system. Massive size-magnesium ammonium phosphate

 

Clinical Course

·         Renal pelvis stones-few symptoms or renal damage

·         Ureteral passage-intense pain-renal or urethral colic-paroxysms of flank pain radiating toward the groin

·         Gross hematuia

·         Urinary flow obstruction

 

Tumors of the Kidney

·         Renal cell carcinoma—most common

·         Wilm’s tumor—kids

 

Renal cell Carcinoma

·         Adenocarcinoma from tubular epithelial cells

·         80-90% of kidney cancer

·         2% of cancers overall

·         men 2;1—ages 60-70

·         associated w/ von Heippel-Lindau syndrome (VHL)

 

Clinical triad

·         painless hematuria, long standing fever, dull flank pain

·         usually large before detection

·         polycythemia in 5-10% (elaboration of erythropoietin by renal tumor)

·         metastasis-lungs and bone

 

Wilm’s Tumor

·         major cancer in children—ranks 3rd for <age 10

·         may arise sporadically or be familial

·         deletion of short arm of chromosome 11 w/loss/mutation of cancer suppressor gene

 

Bladder Tumors

·         most common among these

·         cause more deaths than kidney

1.        papilloma-rare and benign

2.        transitional cell carcinomas-grades I-II-III

·         ranges from noninvasive to invasive, well-differentiated to anaplastic

3.         squamous cell carcinoma-only 5%

4.        in situ

 

·         painless hematuria

·         bladder Men 3:1—ages 50-70 yrs

·         exposure to beta-naphthylamine—increase50x more common

·         cigarette smoking, chronic cystitis, schistosomiasis of bladder, certain drugs (cyclophophamide)

·         stubborn

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