12. Renal Pathoma Flashcards by Trevor Johnson

what artery does horse shoe kidneys get stuck on?

inferior mesenteric artery

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PKD

inherited defect

Bilateral Enlarged kidneys due to cysts in renal cortex and medulla

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Autosomal Recessive PKD

what are the clinical signs

usually presents in infants.

Renal failure, HTN, Hepatic fibrosis

Potters Syndrome

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Autosomal Dominant PKD

ADPKD (ADult)

where is the mutation?

How do they present?

What other d.o’s are they associated with?

The mutation is in PKD1(85%) or PKD2

present with Flank pain, hematuria, HTN, Renal failure(Duh)
Berry aneurysm, hepatic cysts, mitral valve prolapse

(cysts in the liver, brain, and kidney)

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Medullary cystic Kidney Dz

what will the kidneys look like?

inherited defect of Cysts in the collecting ducts

Shrunken Kidneys

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Azotemia

Increase in urine BUN and Creatinine

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Acute Renal Failure

Abrupt decline in renal function measured by Azotemia (Increase in BUN and Creatinine

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Pre-renal azotemia

Intra-renal failur

Post-real Azotemia

Decrease in Renal BLood Flow
-*Acute Tubular Necrosis
-*Outflow Obstruction

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Pre-renal azotemia

Due to decrease in Renal blood flow (Hypotension)
Increased BUN/Creatinine Ratio

(BUN is absorbed, creatinine is not)

Hypotension so the kidney is tryna hold onto whatever it can to conserve volume

BUN/Creatinine ration >20

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Acute Tubular Necrosis

(intra renal failure)

Whats seen in the urine casts?

what are serum and lab findings

What are the two ways to get it?

Injury and necrosis of Tubular epithelial cells. These necrotic cells plug the tubule which obstructs and Decreases GFR

Muddy brown casts
bc the epithelial cells are fucked you get messed up (decreased) resorption of:

BUN which means BUN:CR is

Na which means FeNA >2%

Inability to concentrate urine — Osmalitly

-Ischemic & Neprotoxic

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Ischemic Acute Tubular Necrosis (ATN)

which parts are most often fucked up

Decreased Blood supply results in necrosis of tubules

-PCT(proximal tubule) and Thick ascneding limb

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Nephrotoxic Acute Tubular Necrosis (ATN)

What parts get fucked up?

what agents are nephrotoxic?

Necrosis of tubule cells due to toxic agents

only PCT (proximal tubule)
Aminoglycosides, heavy metals, Myoglobinuria (Crush injury), Ethylne Glycol, Radio contrast

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Acute Interstitial Neprhitis

which drugs cause this?

what is a giveaway if you see it in the urine

what can it lead to?

Intra renal ARF (acute renal failure)

Drug induced hypersensitivity reaction

NSAIDs, Penicillins, Diuretics
Eosinophils in the urine
can lead to Renal Papillary Necrosis

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Renal Papillary Necrosis

clinical symptoms
causes (SAAD Papa)

Necrosis of Renal papillae

Gross Hematuria and flank pain

-causes: Sickle cell, Acute pyleonephritis, Analgesics(Nsaids), Diabetes Mellitus

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NephrOtic Syndrome

Whats the main hallmark of this?

what all lost becasue of it

List all 6 dz that leads to this

PrOteinuria (>3.5g/day). due to any damage of the GFR barrier

Loss of Albumin* which causes *EDEMA

Loss of immunoglobulins –> increased infections

Loss of Antithrombin III(ATIII) –> Hypercoag

Hyperlipidemia- frothy urine

Minimal Change Dz, Focal segmental glomerulosclerosis (effacement of podocytes)
Membranous Nephropathy, MPGN (deposition of immune complexes)
Diabetic Glomerulonephropathy, Amyloidosis

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Minimal Change Dz (MCD)

who gets it

what causes this which can be seen on EM

which protein do you lose?

treatment?

most common cause of NephrOtic syndrome in kids

-effacement of foot processess (podocytes) due to cytokines

you only lose albumin

-tx: Steroids

Focal Segmental Glomerulosclerosis

Who usually gets this?

What is it associated with

what what it look like on histo

whats special about the treatment and what can it progress to

most common cause of NephrOtic syndrome in Hispanics and Blacks
HIV, Heroine, and Sickle cell
-*Focal (not all glomeruli) Segmental (only part of affected glomeruli) Slcerosis (deposition of pink collagen)
poor response to steriods and can progress to chronic renal failure

Membranous nephropathy

who gets it

whats it associated with

whats it look like on histo due to?

Most common cause of NephrOtic syndrome in Caucasian adults

-Hep B/C, Solid Tumors, SLE

Very thick membranes due to immune complex depositions

-Spike and Dome on EM

Membrano-Proliferative Glomerulonephritis

what does it look like on stain

divided into two types. explain them and what they are associated with

Thickening of the glomerular membrane due to deposits of Immune complexes

Tram Track appearance because the membrane gets split by the mesangial cells

Type 1: Subendothelial deposits. Hep B/C
Type 2: Basement Membrane deposits. C3 Nephritic factor (activates complement by stablizing C3 convertase)

Where are the immune deposits in MPGN (membranous proliferative glomerulonephritis) and Membranous nephropathy

MPGN type 1: Subendothelia

MPGN type 2: basement membrane

Membranous: subepithelial

Diabetic Glomerulonephropathy

What does the diabetes do? how to treat it

what kind of nodules will you see?

cause of NephrOtic syndrome

High blood sugar leads to Nonezymatic glycosylation of GBM

leads to leaky and thickening

also increase in GFR due to constriction of efferent arteries (can be treated with Ace inhibitors

kimmelstiel-wilson nodules

Amyloidosis

what will stain show?

Cause of NephrOtic syndrome

Kidney is the most common organ of Amyloid deposition

Congo Red stain shows apple-green birefiringence

NephrItic Syndrome

whats the hallmark finding

what causes the finding

What are the different types

-_Glomerular *I*nflammation and bleeding_ that leads to hematuria and RBC casts in urine

limited proteinuria (

deposition of immune complex triggers inflammation
Types: Post Strep Glomerulonephritis, Rapidly progressive glomerulonephritis (Crescentic), IgA nephropathy

Post Strep GlomeruloNephritis (PSGN)

when does it occur and the virulence factor

Clinical presentation

-whats it look like on histo

how do you treat it?

Main cause of NephrItic Syndrome

occurs after group A, ß hemolytic strep infection of skin or throat
-M protein*
-Periorbital Edema, Cola Urine, Hypertension*
-*“Lumpy bumpy Hump” GRANULAR on Basement membrane
it usually resolves on its own

Rapidly Progressive Glomerulonephritis (Crescentic) what are the crescents made of? What will the Immunoflorescence look like?

A cause of Nephr**I**tic syndrome that progresses to renal failure in *weeks to months* Crescents in the glomerulus -crescents consist of Fibrin and plasma proteins Linear: Antibodies to basement membrane = **Goodpasture syndrome**

IgA Nephropathy (Berger Dz) when do you usually get it?

Cause of Nephr**I**tic Syndrome **IgA** immune complex deposition in Mesangium of Glomeruli Concurrently with Respiratory or GI Tract infection (the IgA gets secreted by mucosal linings)

Alport Syndrome

cause of Nephr**I**tic syndrome inherited (X-linked) defect in **Type 4 collagen** **-**thinning and slpitting of the GBM isolated hematuria Eye problems, ear problems kidney problmes, cant see, cant pee, cant hear a bee

Urinary Tract infection whos at higher risk whats in the lab findings which bacteria cause these? what if you have a negative culture

Infection of urethra, bladder (cystitis), or kidney most commonly ascending infection, women are more risk due to shorter urethra + Leukocyte esterase, + nitrites, urine culture \>100k - *E Coli \> Staph saprophyticus \> Klebsiella \> Proteus mirabilis* (pee has ammonia scent) - negative culture suggests *Neisseria gonorrhoaeae / Chlamydia*

Pyelonephritis (acute) how does it present? whats in the urine

Infection of the kidney (usually due to ascending infection) -fever, flank pain (**Costovertebral angle**) WBC casts in urine

Pyelonephritis (Chronic) which abnormality usually causes this what does this look similiar to/mimic on histo

when you get acute pyelonpehritis a bunch and you get atrophy and fibrosis of the tubules -vesicoureteral reflux (relfux back into the ureters up to the kidney) cortical scarring with blunted calyx "thyroidization"

Kidney stones symptomes what kind of stones are there and shape/associations/tx

Colicky pain with unilateral flank tenderness, hematuria - Calcium oxylate (most common): envelope,chrons dz, tx=*HCTZ* - Amonium magnesium phosphate: coffin, ureas(+) organisms: Proteus or Klebsiella tx=surgically remove (**Staghorn**) - Uric acid: rhomboid, acidic ph/hot climate/gout(duh), tx=Allopurinol - Cysteine: seen in children, genetic defect where you dont reabsorb Cysteine, **S**taghorn

Renal Cell Carcinoma classic symptoms what can it be associated with what does it look like grossly what mutation leads to this and which chromosome is it on

- hematuria, palpable mass, flank pain - associated with paraneoplastic syndromes (ectopic EPO, ACTH, PTHrP, renin) - golden yellow mass + clear cell Loss of **von hippel-lindau** (**VHL**) suppresor gene on Chromosome 3 smoking

Wilms Tumor how does pt present which mutation? what syndrome can this be a part of?

Most common kidney tumor in kids - blastema - Large **palpable unilateral flank mass**, hematuria, HTN - Loss of function of **WT1** or **WT2** **WAGR**- **W**ilms, **A**niridia(no iris), **G**U malformations, mental **R**etardation