Renal - Pathology Flashcards
inferior mesenteric artery root implicated in this congenital problem
horseshoe kidney
most common congenital renal anomaly
Bilateral renal agenesis 1 problem w/3 complications
Also known as
oligohydraminos
- > lung hypoplasia, flat face w/ low set ears, developmental extremity problems
- potters syndrome
Unilateral renal agenesis complication
hypertrophy and later hyperfiltation later on leading to renal failure risk
Noninherited congenital renal malformation characterized by cysts
what histology seen
dysplastic kidney (usually unilateral)
Collagen adjacent to cysts
inherited defect w/ cysts in the renal cortex and medulla
Polycystic kidney disease
infantile and adults
inherited defect w/ cysts in the medullary collecting duct
Seen grossly
Medullary cystic disease (Auto Dom)
shrunken kidneys
ADPCK presents as (3)
associated w/(3)
Adults w/ HTN, hematuria and worsening renal failure
berry aneurisms
hepatic cysts
mitral valve prolapse
Infantile PCKD presents as
Auto recessive
presents as renal failure and infantile HTN
also have hepatic fibrosis w/ hepatic cyst formation
Acute renal failure seen as
Azotemia and oliguria seen w/in days
Divided into pre renal, post renal and infrarenal
Azotemia def
increased BUN and Creatinine in serum
Prerenal azotemia due to?
See:(3)
BUN:Creatinine
FENa ?
Osm?
decreased blood flow
See decreased GFR, azotremia and oliguria
BUN:Creatinine >15, low flow means BUN can resorb, renin aldosterone working to increase BUN level
FENa 500 mOSM/kg - tubes are fine
Postrenal azotremia ude to
See (3)
BUN:Creatinine
FENa
Osm
obstruction downstream
See low GFR, azotemia and oliguria
Depends:
Early
- BUN:Cr >15, BUN forced into serum w/ backpressur
-FENa 500 mOsm, tubes fine
Late - tubular damage - Can’t resorb as well
-BUN:Cr 1%
Osm <500mOsm
Intrarenal Tubular necrosis is
Types(2)?
Problem w/in the kidney(block) usually resulting in BUN:Cr < 15 ( overall increase in BUN and creatinine)
FENa >1%
Osm <500 mOSM
Acute Tubular Necrosis - tubes
Acute interstitial nephritis - surrounding tissue
Causes of Acute tubular necrosis (2)
Ischemia
- low blood supply, preceded by pre renal azotemia
Nephrotoxic
-aminoglycosides, heavy metals. myoglobinuria(crush injury), ethylene glycol, radio contrast die, urate (tumor lysis syndrome(
Brown granular cases in urine seen in
necrotic cells plugging the tubule in acute tubular necrosis
Tumor lysis syndrome
What is it and prevention?
acute leukemia treated w/ chemo -> increased nuclear material in the blood
Rx w/ hydration and allopurinol
Acute interstitial nephritis Due and causes (3)
Drug induced hypersensitivity of surrounding tissue
NSAIDs, penicillin and diuretics
Clinical features of acute tubular necrosis (3)
oliguria w/ brown granular casts
elevated BUN and Creatine
Hyperkalemia(reduced excretion) w/ metabolic acidosis (decreased organic acid excretion)
eosinophils seen in urine?
acute interstitial nephritis
Renal papillary necrosis
Presentation(2) and causes (4)
gross hematuria and flank pain w/ necrotic renal papillae
Due to: chronic analgesic use, DM, Sickle cell severe acute pyelonephritis
Nephrotic syndrome = what seen
4 complications
> 3.5 g.day filtered
leading to
hypoalbuminemia -edema
hypogammaglobinemia - infection risk
hypercoaguable - loss of anti thrombin III
Hyperlipidemai and hypercholesterolemia - fatty casts in urine (liver wants to thicken blood)
Most common syndrome in kids
Associated w/
minimal change disease
Hodgekin lymphoma - due to cytokine release leading the change
effacement of foot processes on electron microscopy (2)
Rx?
minimal change disease or focal glomerulosclerosis
Steroids, great response in KIDS,
not adults w/ FSGS -> renal failure
HIV, heroin and sickle cell associated nephrotic syndrome
Focal semental glomeulosclerosis
Usually idiopathic
MOST COMMON - hispanic and African americans
Membranous nephopathy is associated w/ (5)
used to be most common - usually idiopathic
HepB HepC solid tumors SLE - (diffuse prolifererative glomerulinephritis more common) Drugs (NSAIDs and penicillamine)
Thick glomeruli basement membrane on H and E w/ immune complex deposits (granular) (2)
Membranous nephropathy
Membranoproliferative glomerulonephritis
(can be nephritis too)
Deposits go to all 3 areas
- subepithelial - membranous nephropathy
- subendothelial - Type I membranoproliferative gom
- BM - type II membranoproliferative glom
Spike and dome appearance EM
Membranous nephropathy -
Podocytes like to be on BM and will lay down BL over subepithelial deposits
Tram Track appearance on Em
Associated w/
Membranoproliferative Glomerulonephritis Type 1
(HBV and HCV)
Mesangial cells adjacent proliferate w/ sub endothelial deposits and tries to split deposit w/ increased cytoplasm -> tram track
Associated w/ C3 nephritic factor
What is It?
membranoproliferative glomerulonephritis Type II
intramembranous deposit (BM)
Antibodies stabilize C3 convertase instead of destroying it -> hyperactive complement activation inflammation and decreased C
nonenzymatic glycosylation of vascular BM -> hyaline arteriolosclerosis
Diabetes mellatis nephrotic syndrome
Kimmelstiel Wilson nodules
Sclerosis of the mesangium seen in DM nephrotic syndrome
Protective agent in DM nephrotic syndrome
ACE inhibitors
Prevents further clamping of the efferent arteriole and associated hyper filtration (selected in the disease progression)
Nephrotic syndrome w/ apple green birefringence
deposits are what type and where?
Systemic Amyloidosis involves kidney most commonly
deposits in the mesangium
AL seen from original cause multiple myeloma
6 types of nephrotic syndrome
Minimal change disease
Focal segmental glomerulosclerosis
Membranous Nephropathy Membranoproliferative Glomerulonephritis (nephritic as well)
Diabetes Mellitus
Systemic Amyloidosis
Nephritic syndrome characterized by(2)
Also see
glomerular inflammation(hyper cellular) and bleeding
limited proteinuria periorbital edema and HTN
RBC casts
Hypercellularity in nephritic syndrome is due to
Immune complex deposition and activation of complement -> C5a recruitment of PMNs
Types of nephritic syndrome(5)
Membranoproliferatve glomerulonephritis poststreptococcal glomerulonephritis rapidly progressive glomerulonephritis - Linear -Granular - Negative IF IgA nephopathy - Berger disease Alport Syndrome
sub epithelial humps seen on EM post sore throat
More common in?
Poststreptococcal glomerulonephritis
2-3 weeks post sore throat or impetigo infection
(group A beta hemolytic strep pharynges)
More common in kids, mote dangerous in adults -> rapidly progressive glomerulonephritis
Sub endo -> sub epi (humps)-> goes away- passes (supportive Rx)
Crescents in Bowmans space are made of what?
Pathology called?
Made of fibrin and macrophages
Nephritic syndrome - Rapidly Progressive Glomerulonephritis
3 immunofloresent patterns seen in Rapidly progressive glomerulinephritis
Linear - Anti BN
Granular - Immune complex deposit
Negative IF - no immune showing
SLE associated nephritic syndrome
diffuse proliferative glomerulonephritis
a nephritic syndrome (rapidly progressive glomerulonephritis) w/ granular deposition
Ab against collagen in the glomerular and alveolar BM
Goodpasture Syndrome
Hematuria and hemoptysis
sub endothelial deposition of immune complexes in adults that does not get better
poststroptococcal glomerulonphritis (granular)
negative IF and C-ANCA nephritic syndrome
3 targets
Wegeners
lung, kidney, nasopharynx
2 p- ANCA disease w/ negative IF nephritic syndromes
Churg Strauss
Microscopic polyangiitis
Churg Struass has 3 differences from microscopic polyangiitis
granulomatous inflammation
eosinophilia
asthma
Most common nephropathy world wide w/ deposition in the mesangium post mucosal infection
IgA nephropathy
episodic gross or microscopic hematuria post illness progressing to renal failure
sensory hearing loss, ocular disturbance and isolated hematuria
All due to
Alport syndrome
Inherited defect in type Iv collagen
X linked
gold standard in cystitis
Other labs
> 100,000 colony forming units
positive leukocyte esterase and nitrites (from bacteria converting nitrates)
Cloudy urine
Presentation of Cystitis(4) and notable absence
dysuria
frequency
urgency and suprapubic pain
absence of systemic signs
Etiology of cystitis (top 5)
E coli Staph saprophyticus - esp if sexually active Klebsiella pneumoniae Proteus miabilis - alkaline urine Enterococcus faecalis
Sterile urine w/ symptoms of cystitis/urethritis suggestive of
have pyuria
Chlamydia tachomatis or Nesseria gonorrhoea
Pyelonephritis noteable addition in presentation over cystitis (4)
fever
flank pain
WBC casts
lekocytosis
Chronic pyelonephritis risk increases w/(2)
vesicoureteral reflux - kids
obstruction (BPH/cervical carcinoma) - adults
Interstitial fibrosis and atrophy of the tubules -> cortical scarring w. blunted calyces called?
multiple bouts of pyelonephritis -> chronic pyelonephritis
Charactersitically scarred in vesicoureteral reflux chronic pyelonephritis
upper and lower poles
esosinophilic proteinaceous material resembling thyroid follicles
Atrophic tubules in chronic pyelonephritis
Waxy casts also
Presentation of nephrolithiasis(3)
Colicky pain
Hematuria
unilateral flank tenderness
Common causes of chronic renal failure(3)
DM
HTN
Glomerular disease
Uremia in chronic renal failure see (7)
increased nitrogenous waste products in the blood->
nausea anorexia pericarditis platelet dysfunction - low adhesion and aggregation encephalopathy w/ asterixis deposition of urea crystals in skin
Clinical Features of Chronic renal disease (6)
Uremia Na and water retention Hyperkalemia and Metabolic alkalosis Anemia - low EPO Hypocalcemia - Renal osteodystophy
Erythropoeitin made by?
renal peritubular interstitial cells
Hypocalcemia in chronic renal failure due to ?
decreased 1 alpha hydroxylation of VIt D by proximal renal tubule cells
and hyperphosphatemia
Bone destruction in chronic renal failure is called what and due to what 3 things?
Renal ostrodystrophy
osteritis fibrosis cystica -> hypocalcemia and secondary hyperparathyroidism
osteomalacia - low Vit D function
osteoperosis - Ca leached from bone to buffer metabolic acidosis
Crohn’s has this renal association?
Due to?
Calcium oxalate stones
Due to increased reabsorbtion of oxalate and precipitation in the
Rx for most common cause of stones in kidneys
hydrochlorothiazide
Ca sparing dieuretic for Ca oxalate stones
Alkaline urine and infection -> this?
Major complication
Ammonium magnesium phosphate stones and urease positive organisms (Proteus or Klebsiella)
Staghorn calculi
Only radiolucent stone
Due to?
Uric acid
Seen in gout patients and hyperuricemia - > leukemia or myeloproliferative
Risk factors for Uric acid stone (3)
hot arid climate
low urine volume
acidic pH
(Rx w/ hydration and alkalination w/ bicarb)
Inherited renal stone risk?
Cystein stones
w/ cystinuria - defect of tubules and decreased reabsorption of cysteine
Also staghorn calculi
Harmatoma w/ blood vessels, smooth muscle and adipose tissue - renal neoplasia
Increased in?
Angiomyolipoma
Tuberous sclerosis
Classic triad of renal cell carcinoma
Hematuria
palpable mass
flank pain
- rare, usually hematuria
See a patient w/ reactive polycythemia or cushing syndrome or hypercalcemia or HTN w. hematuria?
Renal cell carcinoma
Has a lot of paraneoplastic potential
Left sided varicocele w/ hematuria
renal cell carcinoma
Most common subtype of renal cell carcinoma
clear cell
Grossly see a yello mass
Loss of VHL(3p)
Renal cell Carcinoma
A tumor suppressor gene
increased IGF-1 and HIF as a result
2 pathogenesis of renal cell carcinoma and associated epidemiology
Sporadic - seen in adult (>60) smokers unilaterally
Hereditary seen in younger adults bilaterally
Von hoppel Lindau disease - due to
Risks(2)
AD disease w/ inactivation of VHL gene
hemangioblastoma of cerebellum and renal cell carcinoma
METS of renal cell Carcinoma (2)
Nodes located?
Lungs and bone
w/ renal vein involvement
retroperitoneal
Blastema implicated in
what is it
Wilms Tumor
immature kidney mesenchyme
3 year old kid w/ hematuria, HTN an large unilateral flank mass
Wilms tumor
HTN w/ renin
most common malignant
WT1 gene
Tumor suppressor gene implicated in Wilms tumor
90% sporadic
WAGR syndrome(4)
due to?
wilms tumor
Aniridia
genital abnormalities
mental and motor retardation
-deletion of WT1 (llp13)
Denys Drash syndrome(3)
- due to ?
Wilms tumor
progressive renal disease
male pseudohemaphroditism
mutations in WT1
Beckwith Wiedmann Syndrome(4)
-due to?
Wilms tumor
neonatal hypoglycemia
muscular hemihypertrophy
organomegaly - tongue
WT2 gene cluster mutation
Risk factors of Urothelial (transitional cell) carcinoma (5)
Cigarette smoke - main naphthalyamine Azo dye cyclophosphamide long use phenacetin long use
Older adult presents w/ painless hematuria, what must be ruled out
urothelial (transitional cell) carcinoma
Field effect ?
Bladder cancer
multifocal and recur w/ chronic bath of carcinogen
2 types of urothelial carcinoma
- 2 major differences
Flat - starts high grade w/ p53 mutations - Progression
Papillary - starts low grade w/out p53 -> progression
background of squamous metaplasia in the bladder?
3 causes
- predisposes for squamous cell carcinoma
Chronic irritation
- chronic cystitis (old women)
- Schistosoma hematobium (middle east)
- nephrolithiasis
Cancer presents at the dome of the bladder
due to ?
Adenocarcinoma bladder
- malignant prolif of gland cells
often due to Urachal remnant
Other causes cystitis glandularis or extrophy (bladder outside)
string of beads on angiography leading to HTN
fibromuscular dysplasia
seen in middle age women, renal vascular sclerosis
bruits on auscultation
