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Congenital
Horseshoe kidney
o Conjoined at lower pole in lower abdomen
o Inferior mesenteric artery
Renal agenesis
o Absent kidney(s)
Unilateral hypertrophy hyperfiltration renal failure
Bilateral oligohydramnios lung hypoplasia, flat face w/
low set ears, extremity defects (Potter) death
Dysplastic kidney
o Noninherited
o Cysts, cartilage
o Usually unilateral
PKD
o Inherited
o Bilat enlarged kidneys w/ cysts in cortex and medulla
o AR
Infants potter sequence
Congenital hepatic fibrosis portal hypertension, hepatic
cysts
o AD
Young adults
APKD1/2
Berry aneurysm, hepatic cysts, mitral valve prolapse
Medullary cystic kidney disease
o Inherited AD
o Cysts in medullary collecting ducts
o Shrunken kidneys
Nephrotic syndrome
Proteinuria (>3.5 g/day)
o Hypoalbuminemia pitting edema
o Hypogammaglobulinemia incr infection
o Hypercoagulable state loss of ATIII
o Hyperlipidemia and hypercholesterolemia fatty casts
Minimal change disease
o Most common in children
o Normal glomeruli
o Effacement of foot processes
o Selective proteinuria (albumin, not Ig)
o Excellent response to steroids (damage by cytokines from T cells)
Focal segmental glomerulosclerosis
o Hispanics, African americans
o HIV, heroin, sickle cell
o Effacement of foot processes
o Poor response to steroids chronic renal failure
Membranous nephropathy
o Caucasians
o Hep B/C, solid tumors, SLE, drugs (NSAIDs, penicillamine)
o Thick glomerular basement membrane
o Subepithelial immune complex (granular IF) deposition w/ spike
and dome appearance
Membranoproliferative glomerulonephritis
o Thick glomerular basement membrane
o Tram track appearance
o Immune complex deposition
Type I subendothelial; HBV/HCV
Type II (dense deposit disease) intramembranous; C3
nephritic factor (autoAb stabilizes C3 convertase)
complement overactivation, low C3
o Poor response to steroids chronic renal failure
DM
o High serum glucose nonenzymatic glycosylation of BM
hyaline arteriolosclerosis
o Efferent arteriole constriction high GFR microalbuminuria
nephrotic syndrome
ACE inhibitors slow hyperfiltration damage
o Sclerosis of mesangium Kimmelstiel-Wilson nodules
Systemic amyloidosis
o Amyloid in mesangium
o Apple-green birefringence under polarized light w/ Congo red
staining
Nephritic syndrome
Inflammation and bleeding
Limited proteinuria
RBC casts and RBCs in urine
Hypercellular inflamed glomeruli
Immune complex deposition complement
o C5a neutrophils damage
PSGN
o Group A beta-hemolytic streptococcal infection of skin (impetigo) or
pharynx
o M protein virulence factor
o Presents 2-3 weeks after infection
o Hypercellular inflamed glomeruli
o Subepithelial immune complexes (granular IF)
o Treatment is supportive
Rapidly progressive glomerulonephritis
o Crescents in Bowman space fibrin and macrophages
o Linear IF
Anti-GBM Ab
Against collagen in glomerular and alveolar BMs
Goodpasture syndrome
Hematuria and hemoptysis
Young adult males
o Granular IF (immune complex deposition)
PSGN
Diffuse proliferative glomerulonephritis
Subendothelial
Most common renal disease in SLE
o Negative IF (pauci immune)
Wegener granulomatosis
C-ANCA
Microscopic polyangiitis
P-ANCA
Churg-Strauss
P-ANCA
Granulomatous inflammation, eosinophilia, asthma
IgA nephropathy
o IgA in mesangium
o Hematuria w/ RBC casts following mucosal infections
(gastroenteritis)
Alport syndrome
o X-linked
o Type IV collagen
o Thinning and splitting of GBM
o Hematuria, sensory hearing loss, ocular disturbances
Nephrolithiasis
Urinary solute as stone
High concentration of solute in urinary filtrate, low urine volume
Hematuria and unilateral flank tenderness
Calcium oxalate/phosphate stones
o Most common
o Hypercalciuria
o Crohn disease
o Trt HCTZ
Ammonium Mg Phos
o Second most common
o Urease positive organisms infection
Proteus, Klebsiella
Alkaline urine stone formation
o Staghorn calculi
o Trt surgical removal and antibiotics
Uric acid
o Radiolucent
o Hot arid climates, low urine volume, acidic pH
o Gout, hyperuricemia in leukemia or myeloproliferative disorders
o Trt hydration and alkalinization of urine (potassium bicarb)
o Allopurinol gout pts
Cysteine
o Children
o Cystinuria decr reabsorption of cysteine
o Staghorn calculi sometimes
o Trt hydration and alkalinization of urine
Renal neoplasia
Angiomyolipioma
o Hamartoma blood vessels, smooth muscle, adipose tissue
o Incr frequency in tuberous sclerosis
Renal cell carcinoma
o Malignant epithelial tumor from tubules
o Hematuria, palpable mass, flank pain
Paraneoplastic (EPO, renin, PTHrP, ACTH)
Left sided varicocele
o Yellow mass
o Clear cell cytoplasm
o VHL (3p) tumor suppressor gene incr IGF-1 (promotes growth)
and incr HIF txn factor (incr VEGF and PDGF)
o Hereditary or sporadic
Sporadic adult males; single tumor in upper pole of kidney;
smoking risk factor
Hereditary young adults; bilateral
Von Hippel Lindau disease AD disorder assoc w/
inactivation of VHL incr risk fo hemangioblastoma
of cerebellum and renal cell carcinoma
Wilms tumor
o Malignant kidney tumor comprised of blastema (immature kidney
mesenchyme), primitive glomeruli, tubules, stromal cells
Most common malignant tumor in children 3 yrs old avg
o Large unilateral flank mass w/ hematuria and HTN (renin secretion)
o Most sporadic
WAGR syndrome wilms tumor, aniridia, genital
abnormalities, mental/motor retardation; deletion of WT1
tumor suppressor gene (11p13)
Denys-Drash syndrome wilms tumor, progressive renal
disease, male pseudohermaphroditism, WT1 mutations
Beckwith-Wiedemann syndrome wilms tumor, neonatal
hypoglycemia, muscle hemihypertrophy, organomegaly (incl
tongue), WT2 mutations (11p15.5