12. Renal Pathoma Flashcards
what artery does horse shoe kidneys get stuck on?
inferior mesenteric artery
PKD
inherited defect
Bilateral Enlarged kidneys due to cysts in renal cortex and medulla
Autosomal Recessive PKD
what are the clinical signs
usually presents in infants.
Renal failure, HTN, Hepatic fibrosis
Potters Syndrome
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)
Medullary cystic Kidney Dz
what will the kidneys look like?
inherited defect of Cysts in the collecting ducts
Shrunken Kidneys
Azotemia
Increase in urine BUN and Creatinine
Acute Renal Failure
Abrupt decline in renal function measured by Azotemia (Increase in BUN and Creatinine
Pre-renal azotemia
Intra-renal failur
Post-real Azotemia
- Decrease in Renal BLood Flow
- -*Acute Tubular Necrosis
- -*Outflow Obstruction
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
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
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
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
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
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
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
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 NephrItic 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 NephrItic 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 NephrItic 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, Staghorn
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- Wilms, Aniridia(no iris), GU malformations, mental Retardation