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Pathology of the Kidney (parts 1 & 2)


What form of nephropathy is a common response to tumors?
membranous glomerulonephropathy

What is the hallmark sign of Minimal Change Disease (aka lipoid nephrosis)?
flattening of podocyte foot processes seen via electron microscopy

What is the most common cause of nephrotic syndrome in African Americans?
FSGC, focal segmental glomerulosclerosis
also commonly seen in AIDS pts

What is the most common cause of chronic renal failure in the US?
diabetic nephropathy

What is the other name for nodular glomerulosclerosis in diabetics?
Kimmelsteil-Wilson dz

What's the most common cause of nephritic syndrome worldwide?
IgA nephropathy, aka Berger's Dz, ie: immune complexes jam up glomerulus
most common reason for IgA overburden: group A beta-hemolytic streptococcus

People with which food sensitivity are more likely to get IgA Nephropathy?
gluten sensitivity, celiac disease

If the Ab's seen in the glomerulus are IgG, what is the disease likely to be?
lupus, SLE

Which renal syndrome is associated with RBC casts in the urine and hypertension?
nephritic syndrome

What are the crescents that give RPGN the name "crescentic glomerulonephritis"?
epithelial cells with fibrin deposits in crescent shape around glomerular capillaries

What are the top three causes of rapidly progressive glomerulonephritis?
Wegeners, SLE, Goodpasture's

Which of the top 3 causes of RPGN may present with may present with purulent OM, severe sinusitis, conjunctivitis and hemorrhaging gums?
Wegener's
renal pathology is combo of focal and segmental GN w/ granulomatous vasculitis of renal vessels

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What do you call the capillary tuft surrounded by the Bowman's capsule?
glomerulus

How much kidney function is lost before there are symptoms?
75% or more

What space are the kidneys in?
retroperitoneal

Which vertebra is closest to the upper pole of the kidneys?
12th

Which kidney is lower?
right

What's a normal size and weight for kidneys?
4-5" long, 2.5" wide, 5oz in weight

How much of the cardiac output goes to the kidneys?
20-25%, or approximately a liter/minute
1500 L/day

How much urine is usually formed and excreted per day?
about a liter

What are the six main functions of the kidneys?
1) excrete metabolic waste
2) regulate electrolyes
3) acid-base balance
4) activate vitamin D
5) secrete renin (control BP)
6) secrete erythropoetin (signal RBC manufacture)

What is the structural & functional unit of the kidney?
nephron, consisting of glomerulus, PCT, loop of henle, DCT, collecting duct

Name the arterioles that supply and empty the glomerulus?
afferent and efferent respectively

What is used to estimate kidney function?
GFR, glomerular filtration rate
calculated based on???

What stain is used to see the basement membranes of the glomberulus by microscope?
PAS stain, protein acid shift

What are five congenital renal anomalies?
agenesis or one or both (of one: 1/4,000-7,000, may be ASx)
hypoplasia (usudt renal aa crimp, small one prone to infx/scarring, other-->hyperplastic)
horseshoe kidney (fusion usu at lower pole, oft ASx, may have infx dt urinary stasis)
ectopic kidney (usu just one, mc in lower abd or pelvis, short ureter-->more infx risk)
duplicate ureter

What symptoms are usually found with simple cysts?
none
cysts are avascular, serous fluid filled
if burst no bleeding just fluid expulsion
very large cyst may impact renal fx

What's the difference between adult and childhood polycystic kidney disease? (PKD)
adult PKD is autosomal dominant (assoc w/ liver cysts, onset age 20-30)
child PKD is autosomal recessive (may cause fetal demise)

What are the complications of PKD?
recurrent kidney infx-->scarring-->renal insufficiency-->renal failure

What is the presumed mechanism for PKD?
abnormal collagen and elastin

What else can cause cysts besides heredity?
dialysis (mechanism not known)

Dialysis pts are at a much increased risk of what complication?
renal cell carcinoma

What is the most common mechanism of glomerular injury?
immune: most commonly antibodies specific to glomerular basement membrane (anti-GBM Abs)
may be trigger by AB's from infx, allergic, drug rxn, SLE or idiopathic
WBC's & inflammation, deposition of immune complexes on epithelial side of basement memb-->
proliferation of mesangial, endothelia and epithelial cells-->
thickening of basement membrane

What is one form of glomerular disease in which NO immune complexes are seen?
lipoid nephrosis, aka minimum change disease or nil disease

What can result from glomerular injury?
nephrotic syndrome
lipiod nephrosis
membranous glomerulopathy
focal-segmental glomerulosclerosis
nodular and diffuse glomerulosclerosis

How do you recognize nephrotic syndrome?
proteinuria > 3gm/day, enough to make the urine frothy (high albumin)
decreased serum proteins (low albumin)
increased serum lipids
generalized edema, dependent, pitting, dramatic presentation in kids
increasing tendency to clot dt increased fibrinogen synthesis
may see RBC's in urine but not always and not many
MCD shows hyaline protein droplets under light microscopy

What is the most common cause of nephrotic syndrome in children?
lipoid nephrosis (90% of all cases)

At what age is the peak incidence of lipiod nephrosis?
age 2-3 years
also accounts for 20-30% of all adult cases of nephrotic syndrome

How is lipoid nephrosis different from the other causes of nephrotic syndrome?
selective proteinuria (mb 4+), albumin is the only protein lost

What are the other names for lipoid nephrosis?
"nil disease", or minimal change disease (MCD)
because glomeruli look fine under light microscopy

What imaging will reveal the characteristic flattening of podocyte processes in MCD?
electron microscopy

What is the most common cause of ADULT nephrotic syndrome?
membranous glomerulonephropathy, aka membranous glomerulonephritis

What causes membranous glomerulonephropathy?
most idiopathic
some dt antigens: infx, drugs (gold, captopril, tx for RA) or toxins
also mb assoc w/ tumors: lung, colon, melanoma, lymphoma

What infections might trigger membranous glomerulonephropathy?
chronic hep B & C
syphilis
malaria
schistosomiasis

What is the microscopic sign of membranous glomerulopathy?
thickened and prominent capillary loops (protein deposition spikes outside tubes)
(epithelial and mesangial cellularity does not usu increase)
positive immunofluorescent microscopy

What is the most common cause of nephrotic syndrome in African-Americans???
FSGS, focal segmental glomerulosclerosis
it's about 15% of nephrotic syndrome in adults and kids

What causes focal segmental glomerulosclerosis?
idiopathic
immune complexes sometimes but not always seen
commonly seen in AIDS pts (dt dz or drugs?)

How do you recognize focal segmental glomerulosclerosis?
non-selective proteinuria
areas of collagenous sclerosis in glormulus

What's the prognosis for focal segmental?
poor:
poor response to steroid therapy
progression to chronic renal failure

What do you call renal disease due to diabetes mellitus?
diabetic nephropathy

What are the findings with diabetic nephropathy?
proteinuria
glucosuria
progressive decrease in renal fx
hypertension and dependent edema in later stages

How can a diabetic minimize their risk of nephropathy?
stabilize/control blood sugar consistently over years

How many years after a diabetic diagnosis does nephropathy usually develop?
10-20 years

What are some of the renal complications of diabetes?
arteriosclerosis
glomerulosclerosis
pyelonephritis
may present as nephrotic syndrome, anemia

What are the histologic changes seen with diabetic nephropathy?
glomerulosclerosis that is nodular, diffuse or mixed
hyaline material in glomerular capillary loops

What is the other name for nodular glomerulosclerosis in diabetics?
Kimmelsteil-Wilson dz

What glomerular changes are seen in advanced diabetic glomerulosclerosis?
lesions may be nodular, diffuse or mixed
atrophy and hyaline degeneration
eventual papillary necrosis-->renal failure

What other autoimmune disease (besides DM or SLE) may cause renal disease?
amyloidosis
may present as primary condition or secondary to multiple myeloma, lymphoma, etc

What does amyloid look like under the light microscope?
pink, eosinophilic and acellular
homogenous, highly refractive, with an affinity for Congo red dye
electron microscopy reveals characteristic linear "fibrils" in the deposits
first desposited in mesangium and capillary walls
later accumulating in glomerular and tubular basement membranes
obliteration of glomerulus

What is the progression of signs in amyloidosis of the kidneys?
initially mild proteinuria
kidneys may enlarge
tongue is thick with dents on sides from teeth
spontaneous ecchymosis on upper eyelids
severe nephrotic syndrome
prognosis is poor: need a transplant

What causes glomerulonephritis?
primary or immune mediated
immune aggravation due to infx (bact, viral, parasit), drugs, systemic dz (SLE, vasculitis), CA

What are the two main types of glomerulonephritis?
proliferative and nonproliferative

What are some examples of nonproliferative glomerulonephritis?
Minimal Change DZ, and Focal Segmental Glomerulosclerosis (MCD and FSGS)

How to people with nonproliferative usually present?
nephrotic syndrome

What are the signs of nephrotic syndrome?
extreme proteinuria (>3gm/day), foamy/frothy
decreased serum proteins
increased serum lipids, fatty casts in urine
generalized edema
possible hematuria but no RBC casts in urine

What's the usual presentation of proliferative glomerulonephritis?
nephritis syndrome, usu dt IgA nephropathy aka Berger's Dz
weeks to years to end stage renal failure (ESFR)
HTN, variable renal insufficiency
proteinuria
RBC casts**
azotemia
oliguria (<400mL/day)

What's the most common cause of nephritic syndrome worldwide?
IgA nephropathy, aka Berger's Dz
immune complexes jam up glomerulus
post resp, GI or GU infx, bact, fung or parasit, food incl: gluten

People with which food sensitivity are more likely to get IgA Nephropathy?
gluten sensitivity
celiac disease

How do you diagnosis IgA Nephropathy?
Bx tissue, immunofluorescent stain

Name the disease involving IgA hypersensitivity vasculitis of vessel walls in skin, kidneys, GI?
Henoch-Schoenlein purpura (HSP)

What are the signs and prognosis of Henoch-Schoenlein purpura?
signs: skin lesions, joint tenderness, hematuria, vasculitis (skin Bx)
usu in kids age 3-8
may be vaccination induced
prog: usu resolves without tx in kids, more sx and sequellae in adults
conventional tx: steroids

If the Ab's seen in the glomerulus are IgG, what is the disease likely to be?
lupus, SLE

What causes the most acute nephritic sydrome worldwide?
group A beta-heolytic strep (strep throat, impetigo)

What are the signs of post-streptococal glomerulonephritis?
1-2 wks after infx see RBCs on UA, possible casts, fever, chills, HTN

What histological evidence of post-strep GN might one find with a kidney biopsy?
under microscope see glomerulolar infiltration by PMNs and macrophages
endothelial and mesangial proliferatione
immunoflourescent studies show granular deposition of IgG, C3 and mesangial fibrin
electron microscopy shows "lumpy" appearance
with diagnostic finding of sub-endothelial "clumps of humps"

What fraction of kids with strep have total recovery and no sequellae?
80-90% or more

What is RPGN?
Rapidly Progressive Glomerulonephritis aka crescentic or necrotic GN
just GN that starts with hematuria, oliguria, HTN
possible fever, myalgia, arthralgia, edema, abdominal pain, fatigue
and goes to renal failure faster
dialysis/transplantation needed for survival

What can increase the risk of RPGN?
vascular disease: vasculitis, polyarteritis
collagen-vascular disease: lupus nephritis, Goodpasture's syndrome, Henoch-Schoenlein purpura
IgA nephropathy
membranoproliferative GN
abscess of any internal organ
history of malignant tumors, blood of lymphatic system disorders
exposure to hydrocarbon solvents (benzene, for example, is in canned soda pop)

What are the crescents that give RPGN the name "crescentic glomerulonephritis"?
epithelial cells with fibrin deposits in crescent shape around glomerular capillaries

What are the organ systems impacted by Goodpasture's syndrome?
kidneys, lungs
present with hemoptysis, hematuria, anemia, pulmonary infiltrates
IgG Ab's to basement membrane, deposition of immune complexes in linear pattern

What's the prognosis with Goodpasture's?
poor
need aggressive tx ith steroids, chemo and plasma exchange
death usudt aspiration of pulmonary hemorrhage

What is Wegener's granulomatosis?
a rare form of vasculitis (20-30/1,000,000)
may have multi-organ involvement
may present with mucosal inflam and ulceration, purulent OM and severe sinusitis, conjunctivitis, scleritis, episcleritis, gum swelling, hemorrhage, ulceration
sinusitis, hemotysis, proteinuria

What are the top three causes of rapidly progressive glomerulonephritis?
Wegeners, SLE, Goodpasture's

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