Obstructive Disease-- Melissa* Flashcards by Leah Stalnaker

Describe how obstruction causes renal pathology; what is the name of the dangerous phenomenon that can ensue?

Obstruction of urine flow–> Infection/ calculi –> HYDRONEPHPHROSIS (dilation of renal pelvis/ calyces; atrophy)

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Define Hydronephrosis:

dilation of renal pelvis/ calyces + atrophy of kidney due to obstruction of urine outflow

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What are some causes of obstruction?

Anything that stops urine flow (Calculi, tumors, BPH, neurogenic bladder, inflammation…)

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How does acute obstruction present? Which size calculi would be most painful if causing obstruction?

painful; small stones are most painful (lodge in ureter)

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How does bilateral complete obstruction present?

oliguria, anuria

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How does unilateral complete or partial obstruction present?

Hydronephrosis or silent

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Which population gets renal calculi most? Are they typically bilateral or unilateral?

Young adult males; typically unilateral

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What are three potential causes of renal calculi?

Familial (inborn error of metabolism)
Supersaturated urine (^conc. stone stuffs; low volume)
Bacterial infection

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Calcium Oxalate PO4 Calculi:

Prevalence? #1 cause? Appearance?

70% all calculi (Most common!)
#1 cause: Idiopathic Hypercalcinuria
Stones are radiopaque octahedron crystals

(O)xalate =(O)ctahedron

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What are some other potential causes of Calcium Oxalate & PO4 Calculi? (4)

All hypercalcemic states and acidic states
Hyperoxaluria (vegetarians)
Hyperuricosuria
Hypocitraturia

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Struvite calculi: 
Prevalence? 
#1 Cause? 
What do crystals look like? 
What classic finding might struvite stones cause? 
How does step love to ask about this?

15-20% all calculi
#1 cause: PROTEUS & Staph (urea splitting bacteria)
*Look for vignette with chronic UTI and ABX use
Crystals: Three to six sided “coffin lids”
After SIX PROTEUS infections, you might be laying in a COFFIN.

IMPORTANT: THESE MAY CAUSE STAGHORN CALCULI!!! ON CT, THESE LOOK LIKE OPACITIES FILLING ALL OF THE RENAL CALYCES. IMPORTANT FOR THIS, MEADOWS, AND STEP!

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Describe how struvite calculi form:

Proteus: Urea–> ammonia–>ALKALINE URINE–> Mg/AmmoniumPO4 salts precipitate

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Uric Acid calculi:
Prevalence? 
#1 cause? 
Acidic vs basic? 
Stone/ Crystal appearance?

5-10% all calculi

# 1 Cause: Hyperuricemia (Gout)
Remember that most chemo drugs cause gout!!!*
Acidic pH
Stones are radioLUCENT
Crystals are flat, 4 sided, lemon shaped

FOUR LEMONs have a lot of ACID.

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Cystine Calculi: 
Prevalence?
#1 Cause? 
Acidic vs. Basic? 
Describe the crystals.

~2% of all calculi

# 1 cause = genetic defects in renal absorption of AA
Acidic pH
Crystals are hexagonal and laminated

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Are benign renal tumors more urothelial or renal cell tumors? Are they usually symptomatic? Found in kids or adults?

More urothelial; usually incidental findings; usually in adults unless genetic disease (tuberous sclerosis); this may just be because adults have more imaging etc –> asymptomatic tumors are naturally less likely to be detected in kids.

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Renal papillary adenoma:
Common cause?
Defining size?

Associated with long term hemodialysis/Cystic disease

- Clearly benign; less than 0.5cm

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What gene mutation is associated with renal papillary adenoma?

Lacks 3p alteration

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Renal Fibroma/Hamartoma:
Macro path (What is defining size)?
Micro path?

Macro: Gray-white nodule less than 1 cm in the renal pyramids
Micro: collagen and fibroblasts trap tubules/ cement them in!

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Angiomyolipoma:
How common are these tumors?
With what disease are these tumors associated?
Describe the macro and micro path.

Rare
Macro: thick walled blood vessels, smooth muscle, fat
Micro: spindle and epithelioid cells
When multiples think Tuberous Sclerosis (AD)

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What are two gene mutations associated with tuberous sclerosis (2)?
What are some other anomalies associated with the disease other than renal angiomyolipomas?

AD LOF mutations:
-Chrom 9, TSC1
-Chrom16, TSC2
Lesions in cerebral cortex, skin, heart etc.

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Oncocytoma:
Population?
Macro and micro path?

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Old Men
Macro: MAHOGANY brown w/ FIBROSIS
Micro: small eosinophilic (pink) cells w/ prominent nucleoli

List the 4 benign tumors of the kidney:

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renal papillary adenoma
renal fibroma/ hamartoma
angiomyolipoma
oncocytoma

Incidental findings, adults, small.

What is a stag horn calculi?

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large stones fill pelvis +/- calyces

Caused by urea splitting bacteria; i.e. proteus/ staph

What is a colic calculi?

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smaller stones passed into ureter = painful; common in young males

Describe the T1-T4 stages of renal malignancy:

T1: LESS than 7cm, kidney only T2: GREATER than 7cm, kidney only T3: Invades veins, adrenals, perinephric tissue T4: Invades Grerota’s fascia and beyond

Two types of malignant tumors of the kidney:

``` Renal cell Ca (renal tubular epi) Urothelial Ca (renal pelvis; urothelium; similar to bladder) ```

``` #1 risk for renal cell ca? #1 population for renal cell ca? Are they typically sporadic or familial? ```

``` #1 risk =TOBACCO #1 population: male smokers Tumors are typically sporadic ```

What is the #1 site for renal cell ca mets?

``` #1 site = LUNG *25% initially present with mets ```

Clinical triad for renal cell ca?

``` Clinical Triad (10%): 1.Costovertebral tenderness 2.Palpable mass 3.Hematuria (intuitive) ```

How are renal cell ca's treated?

Treatment: | Partial or complete nephrectomy (depends on stage)

5 types of renal cell ca + prevalence:

- Clear Cell (80%) - Papillary (under 15%) - Chromophobe (5%) - Collecting Duct - Xp11 Clear cell most common; all others rare.

Clear Cell: Population? Commonly associated genetic disorder? Micro path?

-Adult tumor -Associated with Von Hippel-Lindau Disease (AD) Micro: tumor cells with glycogen + lipid--> clear cytoplasm

What are some manifestations of VHL disease?? (6) | What is the genetic problem?

Von Hippel-Lindau Disease (AD)--> - Retina and Cerebellar hemangioblastomas - Pancreatic, hepatic - renal cysts; Pheochromocytoma Mutation: Deletion of VHL gene on Chrom 3p

Two types of papillary renal cell ca and their genetic mutations:

Sporadic - Trisomy: 7, 16, or 17 - Loss of Y in males Familial -Trisomy 7; MET GOF mutation (TK receptor)

Papillary renal cell ca: Population? With what medical procedure are these tumors associated? Macro and micro path?

- Adult Tumor; commonly associated with dialysis - Macro: Multicentric tumor - Micro: Many papilla; foamy histiocytes; psammoma bodies (Ca++ deposits)

Chromophobe renal cell ca: Population Micro path Common stain used?

- Adult Tumor - Micro: Prominent cell membrane; pale eosinophilic cytoplasm; Perinuclar HALO - Stains blue on Hale’s Colloidal Iron

Collecting duct renal cell ca: Population/ Prognosis? Micro path?

- Aggressive Adult tumor; does not respond to chemo; poor prog. - Micro: Desmoplastic stroma; tubulopapillary architecture; HOBNAIL CELLS (protruding nuclei)

Xp11 renal cell ca: Population Micro path

- Younger patients | - Micro: looks similar to clear cell ca. but w/ papillary architecture (fibroblastic core w/ papillary projections)

Was is the translocation associated with Xp11 renal cell ca?

TFE3 gene translocation--> ^ TFE3 transcription factor

From what tissue do urothelial ca of the renal pelvis arise and to what tumor are they similar? ]

-Arise from urothelium of renal pelvis (like bladder tumors)

Wilms tumor is what kind of kidney tumor?

Urothelial tumor; malignant; in kiddos! | *Rx question; asked about chromosome 11 mutation.*

Wilms tumor: Population Macro and micro path (3 cell types)

- 2-5yoa (#4 pediatric malignancy) Macro: Large round tumor Micro: Poorly differentiated BLASTEMA cells; STROMAL cells; EPITHELIAL cells (3 cell types)

Anaplasstic Wilms tumors are associated with what genetic anomaly? What is the prognosis for these tumors? What is the prognosis for a regular films tumor?

Anaplastic tumors= p53 mutations - Anaplastic tumors can be resistant to chemo and have bad prognosis - Regular wilms tumors have good prognosis with chemo and surgery

WAGR SYNDROME: Genetic mutations (2) Clinical triad

Chrom 11 WT1 gene: Wilms deletion PAX gene: Aniridia deletion Clinical Triad (33% develop Wilms Tumor): - Aniridia (no iris!) - Genital anomalies - Mental retardation

DENYS DRASH SYNDROME: Genetic mutations Clinical triad?

WT1 gene: defective DNA binding properties Clinical triad: - 90% have Wilms Tumor - Gonadal dysgenesis (male pseudohermaphroditism) - Early onset nephropathy w/ diffuse mesangial sclerosis

Bechwith –Wiedmann Syndrome (BWS): Genetic mutations? Salient clinical features (2)?

Chrom 11--> WT2 gene mutation (distal to WT1) Patients have Wilms tumor and organomeagly

Hyper acute Rejection: Timing Gross and micro path Treatment

Min/ Hrs -Gross: Cyanotic, mottled flaccid organ -Micro: Fibrin thrombi, WBCs Clinical: MUST REMOVE KIDNEY

Acute Cellular Rejection: Timing Mediator + micro path Prognosis

Days/Wks -T CELL MEDIATED (CD4, CD8) -Micro: Interstitial mononuclear infiltrate; edema; parenchymal injury Clinical: Responds to prompt ^immunosuppressive therapy

Acute Humoral Rejection/ Rejection vasculitis: Timing Mediator + micro path Prognosis

Days/Wks -B CELL MEDIATED (Igs) -Micro: necrotizing vasculitis; neutro infiltrate; fibrin thrombi; deposition of ***C4d*** (classical complement) Clinical: Treat with B cell depleting agents

Chronic Rejection: Timing Micro path Clinical features and prognosis

Mos/Yrs -Creatinine ^ progressively over 4-6 mos -Micro: glomerular hyalinization; tubular atrophy + chronic inflammation Clinical: does NOT respond to immunosuppressive therapy u

``` Tumor assc with VHL Tumor assc with Tuberous sclerosis Tumor assc with chrom 11 Tumor assc with TFE3 Tumor assc with MET GOF ```

VHL: Clear Cell TS: angiomyolipoma Chrom 11: Wilms TFE3: Xp11 Renal carcinoma MET GOF: Familial Papillary Renal Carcinoma (NOTE: Sporadic is trisomy 7, 16, 17 or Y deletion)

Obstructive Disease-- Melissa* Flashcards

(51 cards)