Glossary of Kidney 2

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Nephritic Syndrome
1. hematuria (red cell casts and smoky brown urine)
2. hypertension
3. oliguria
4. azotemia
Nephrotic Syndrome
1. massive proteinuria
2. hypoalbuminemia
3. generalized edema
4. hyperlipidemia
membranous glomerulonephritis
i. primary cause of nephrotic syndrome (with azotemia)
ii. immune complex disease mostly in teens and young adults
iii. thickened capillary walls and basement membrane
iv. immune complexes in intramembranous an epimembranous locations: “spike and dome” appearance
v. granular immunofluorescence (IgG or C3)
vi. 10% of SLE pts; also associated with hep B, syphilis, malaria infection, drugs (gold or penicillamine), malignancy
vii. sometimes causes renal vein thrombosis
minimal change disease (lipoid nephrosis)
- nephrotic syndrome
- LM: normal glomeruli
- EM: foot process effacement
- most common cause of childhood nephrotic syndrome
- responds well to steroids
- prototype of nephrotic syndrome
- fusing of epithelial foot processes and lipid-laden renal cortices
focal segmental glomerular sclerosis
- nephrotic syndrome
- LM: segmental sclerosis and hyalinosis
- more severe disease in AIDS pts
- similar to minimal change disease, but in older pts
diabetic nephropathy
i. striking increase in thickness of glomerular basement membrane; one of earliest changes in diabetes mellitus
ii. increase in mesangial matrix
(1) diffuse glomerulosclerosis
(2) nodular glomerulosclerosis (Kimmelstein-
Wilson Nodules)
SLE (5 patterns of renal involvement)
- nephrotic syndrome
- LM: wire loop appearance with extensive granular subendothelial basement membrane deposits in membranous glomerulonephritis pattern
acute poststreptococcal glomerulonephritis
- nephritic syndrome
- LM: glomeruli enlarged and hypercellular, neutrophils, "lumpy bumpy"
- EM: subepithelial humps
- IF: granular pattern
- most frequently seen in children; peripheral periorbital edema; resolves spontaneously
- intense inflammatory reaction with many punctate hemorrhages, enlarged hypercellular swollen bloodless glomeruli
rapidly progressive (crescentic) glomerulonephritis
- nephritic syndrome
- LM and IF: crescent moon shape
- rapid course to renal failure from one of many causes
Goodpasture's syndrome (type II hypersensitivity)
- nephritic syndrome
- IF: linear pattern, anti GBM antibodies
- hemoptysis, hematuria
membranoproliferative glomerulonephritis
- nephritic syndrome
- EM: subendothelial humps; "tram track" due to GBM in two layers
- slowly progresses to renal failure
IgA nephropathy (Berger's Disease)
- nephritic syndrome
- IF and EM: mesangial deposits of IgA
- mild disease; often post-infectious
Alport's syndrome
- nephritic syndrome
- split basement membrane
- collagen IV mutation; nerve deafness and ocular disorders
renal amyloidosis
i. subendothelial and mesangial amyloid deposits
ii. criss-cross fibrillary pattern of amyloid by EM
iii. associated with chronic inflammatory disease such as rheumatoid arthritis or plasma cell disorders such as multiple myeloma
minimal change disease (lipoid necrosis)
i. prototypic nephrotic syndrome, seen most in children, responds to adrenal steroid therapy
ii. lipid-laden renal cortices (tubular cells)
iii. glomeruli appear normal
iv. epithelial foot processes are fused
focal segmental glomerulosclerosis
i. similar to minimal change disease, but in older pts
(1) focal distribution
(2) segmental distribution
lupus nephropathy
i. renal component of SLE; severity of this is associated with overall prognosis of SLE pts
(1) Type I: no observable renal involvement
(2) Type II: mesangial form of disease; focal and segmental glomerular involvement with increase in mesangial matrix; slight proteinuria and hematuria
(3) Type III (focal proliferative form): involves less than half the glomeruli
(4) Type IV (diffuse proliferative form): prototype of Lupus nephropathy; involves almost all glomeruli; combined nephrotic and nephritic syndromes; wire-loop abnormality; endothelial cell proliferation; subendothelial immune complex deposition
(5) Type V (membranous form): indistinguishable from primary membranous GN
poststreptococcal GN (acute proliferative GN)
i. prototype of nephritic syndrome
ii. often follows or accompanies infection; nephritogenic strains of group A beta-hemolytic streptococci
iii. complete recovery by almost all children and many adults
iv. immune complex disease where antigen is streptococcal
v. market by several lab abnormalities: urinary red cells and red cell casts, azotemia, decreased serum C3, and increased titers of antistreptococcal antibodies, anti-DNAase B
vi. intense inflammatory reaction in almost all glomeruli
(1) many punctate hemorrhages
(2) enlarged, hypercellular bloodless glomeruli
(3) electron dense humps (subepithelial location)
(4) “lumpy bumpy” immunofluorescence (extremely coarse granular immunofluorescence for IgG or C3)
rapidly progressive (crescentic) GN (RPGN)
i. nephritic syndrome progressing rapidly to renal failure within weeks or months
ii. fibrin deposits in Bowman’s space, parietal epithelial cells in Bowman capsule proliferate; these events form crescents between Bowman’s capsule and glomerular tuft
iii. poststreptococcal etiology, lupus nephropathy, IgA nephropathy are all possibilities when immune complex is deposited (Type II)
iv. (Type I) antiglomerular basement membrane antibodies in 10% of cases; looks like Goodpasture’s
v. pauci-immune type: antineutrophil cytoplasmic antibodies (ANCAs) (Type III)
Goodpasture syndrome (anti GBM disease)
i. antiglomerular basement membrane antibodies directed against glomerular and alveolar basement membranes
ii. linear immunofluorescence (IgG)
iii. clinically manifest by
(1) nephritic syndrome
(2) pneumonitis with hemoptysis
(3) men in mid-20s age group
(4) RPGN crescentic morphology with linear immunofluorescence
focal glomerulonephritis
i. focal and segmental inflammation and proliferation, NOT sclerosis
ii. immune complex disease associated with SLE, SBE, polyarteritis nodosa, Goodpasture syndrome, Wegener granulomatosis, IgA nephropathy
Alport syndrome`
i. hereditary nephritis associated with nerve deafness and ocular disorders
ii. progresses to end-stage renal disease by age 30
iii. irregular GBM thickening with foci of splitting of lamina densa
IgA nephropathy (Berger disease)
i. IgA deposits in the mesangium
ii. benign recurrent hematuria in kids after infection
iii. part of Henoch-Schonlein disease
membranoproliferative GN
i. slow progression to chronic renal disease
ii. GBM thickening and cellular proliferation
iii. reduplication of GBM into 2 layers due to expansion of mesangial matrix into 2 layers; “tram-track” appearance in silver stains
iv. two forms
(1) Type I: immune complex nephritis with unknown antigen and striking tram-track appearance
(2) Type II: more subtle tram-track appearance; irregular electron-dense material deposited within GBM; possibly caused by IgG autoantibody
Infection of the urinary tract and kidney
A. Characteristics
1. more in women than men; structures and pregnancy
2. hematogenous spread vs. ascending infection from vesicoureteral reflux
3. frequently involves normal colon flora such as E. coli
B. predisposing factors
1. obstruction
2. surgery
3. catheters
4. gynecologic abnormalities
C. clinical manifestation
1. urinary frequency
2. dysuria
3. pyuria (neutrophils)
4. hematuria
5. bacteriuria
D. significant findings in acute pyelonephritis (acute infection of renal parenchyma)
1. fever, leukocytosis, flank tenderness, urinary white cells, white cell casts (this last finding is PATHGNOMONIC of acute pyelonephritis)
E. cystitis: pyruria and hematuria, but no white cell casts
acute drug-induced interstitial nephritis
1. penicillin derivatives (methicillin) and other drugs like NSAIDs and diuretics
2. immune etiology
3. acute interstitial renal inflammation
renal papillary necrosis (necrotizing papillitis)
1. ischemic necrosis of tips of renal papillae
2. associated with diabetes mellitus, in which it is related to renal infection and coexisting vascular disease
3. can be catastrophic consequence of acute pyelonephritis
4. associated with long-term abuse of phenacetin and can lead to chronic analgesic nephritis (loss and atrophy of tubules and interstitial fibrosis and inflammation)
acute tubular necrosis
1. most common cause of acute renal failure
2. completely reversible by regeneration of tubular cells in 2 weeks
3. can lead to cardiac standstill from hyperkalemia during the initial oliguric phase
4. most often precipitated by renal ischemia
5. often associated with crush injury with myoglobinuria
6. can be caused by direct injury to proximal renal tubules from mercuric chloride, gentamicin, and several other toxic substances
Fanconi syndrome
a. generalized dysfunction of proximal renal tubules; hereditary or acquired
b. glycosuria, hyperphosphaturia, hypophosphatemia, aminoaciduria, systemic acidosis
a. hereditary; impaired tubular reabsorption of cystine
b. cystine stones
Hartnup disease
a. hereditary; impaired tubular reabsorption of tryptophan
b. leads to pellagra-like manifestations
chronic pyelonephritis
a. coarse, asymmetric corticomedullary scarring and deformity of renal pelvis and calyces
b. early interstitial inflammatory infiltrate in early stages and later interstitial fibrosis and tubular atrophy
c. atrophic tubules contain eosinophilic proteinaceous casts, resulting in thyroidization of the kidney
d. almost always due to chronic urinary tract obstruction and repeated bouts of acute inflammation
diffuse cortical necrosis
A. ischemic infarction of cortices of both kidneys
B. associated with obstetric catastrophes
C. associated with septic shock
D. maybe caused by end-organ vasospasm and DIC
A. diffuse deposition of calcium in kidney parenchyma leading to renal failure
B. due to hypercalcemia from hyperparathyroidism or in milk-alkali syndrome
C. also caused by hyperphosphatemia
calcium stones
80% of all stones; calcium oxalate or calcium phosphate or both; radiopaque; hypercalciuria
1. increase intestinal calcium absorption
2. increased primary renal excretion of calcium
3. hypercalcemia
a. hyperparathyroidism
b. malignancy: osteolytic metastases or ectopic PTH production
c. other causes: sarcoidosis, vitamin D intoxication, milk-alkali syndrome
ammonium magnesium phosphate stones
1. second most common
2. radiolucent
3. facilitated by alkaline urine due to urease positive (ammonia producing) organisms, including Proteus vulgaris or Staphylococcus
4. can form large staghorn (struvite) calculi, ie. casts of renal pelvis and calyces
uric acid stones
hyperuricemia due to gout or increase cellular turnover (leukemia or myeloproliferative syndromes)
adult polycystic kidney disease
1. autosomal dominant, most common inherited disorder of kidney
2. partial replacement of kidney parenchyma by cysts
3. manifest clinically ages 15-30 years
4. associated with berry aneuryism of circle of Willis
5. associated with cystic disease of other organs
6. can be cause of secondary polycythemia
7. clinical manifestations
a. hypertension
b. hematuria
c. palpable renal masses
d. progression to renal failure
infantile polycystic kidney disease
1. autosomal recessive disease
2. multiple cysts evident at birth
major clinical characteristics of uremia
1. azotemia
2. acidosis
3. hyperkalemia
4. abnormal control of fluid volume
5. hypocalcemia (no active vitamin D)
6. anemia (decreased EPO)
7. hypertension (overproduction of renin)
prerenal azotemia
1. decreased renal blood flow leading to increased reabsorption of sodium and water, oliguria, concentrated urine
2. oliguria from decrease renal blood flow leads to increase reabsorption of sodium
3. oliguria from acute tubular necrosis leads to impaired reabsorption of sodium
4. BUN:creatinine ratio is greater than 15 due to lower GFR and increased urea reabsorption
small, asymptomatic, renal tubule cells, precursor to renal carcinoma
hamartoma consisting of fat, smooth muscle and blood vessels; associated with tuberous sclerosis syndrome
renal cell carcinoma
a. most common renal malignancy
b. men aged 50-70, smokers, renal tubules, one of renal poles
c. invades renal veins or vena cava
d. early hematogenous dissemination
e. polygonar clear cells, sometimes vestigial tubule formation
f. triad: flank pain, palpable mass, hematuria (hematuria is common)
g. additional clinical findings
i. fever
ii. secondary polycythemia (from EPO production)
iii. ectopic production of various hormones
Wilms tumor
a. most common renal malignancy of early childhood
b. peak in ages 2-4 years
c. big palpable flank mass
d. histologically varied picture
e. chromosome 11 shortening
f. WAGR complex (Wilms tumor, Aniridia, Genitourinary malformations, mental-motor Retardation)
transitional cell carcinoma
1. most common tumor of urinary collecting system; can occur in renal calyces, pelvis, ureter, or bladder
2. associated with phenacetin abuse in renal pelvis
3. presents with hematuria
4. spreads by local extension to surrounding tissues
5. associated with toxic exposures
a. benzine, naphtylamine, aniline dye
b. cigarette smoking
c. Long-term treatment with cyclophosphamide
squamous cell carcinoma
1. minority of urinary tract malignancies
2. from chronic inflammatory processes such as chronic bacterial infection or Schistosoma hematobium infection
simple (solitary) renal cyst
1. common lesion that occurs in adults; often asymptomatic
uremic medullary cystic disease (nephronophthisis)
1. rare, serious cystic disease affecting older kids
2. cysts in medulla that may result in renal failure
medullary sponge kidney
1. multiple small medullary cysts and impaired tubular function, without renal failure
acquired cystic disease
1. associated with long-term dialysis therapy
2. increase incidence of renal cell carcinoma

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