Robbins Ch. 20 - The Kidney III Flashcards
affect on calyces
only chronic PN and analgesic nephropathy
xanthogranulomatous pyelonephritis
accumulation of foamy macrophages
-proteus infection (weil felix)
drug-induced interstitial nephritis
fever, eosinophilia, rash
-PCN, MCN, rifampin, thiazides, NSAIDs
prep for colonoscopy
acute phosphate nephropathy
-precipitate of calcium P
bence jones
myeloma kidney
-pink blue cast
tophus
mononuclear response with giant cells
-chronic urate nephropathy
medial/intimal thickening, hyalinzation of artery walls
nephrosclerosis
granular kidney, shrinkage
elderly, blacks, DM - increased
malignant nephrosclerosis
BP > 200/120
papilledema, retinal hemorrhage, confusion
hyperplastic ateriosclerosis (onion skinning)
elevated renin levels
renal artery stenosis
curable with surgery
-70% caused by atheromatous plaque
- increased renin - leads to ischemic kidney
- causes HTN
-also - fibromuscular dysplasia (string of beads)
string of beads
fibromuscular dysplasia
bruit on kidney, renin, response to ACE (-)
renal artery stenosis
Tx - 70-80% cured with surgery
E. coli 0157/H7
typical HUS
mutation in factor H
atypical HUS
also factor I and CD46
Tx typical HUS
dialysis - recover in weeks
antiphospholipid syndrome
atypical HUS
also pregnancy, systemic sclerosis, chemo, radiation
ADAMTS13
TTP
-inhibitory autoantibodies
Tx for TTP
plasmapharesis
subendo deposits of fibrion and mesangiolysis
TTP
reduced NO and prostaglandins
prothrombotic
-in TTP/HUS
generalized ischemia to kidney
diffuse cortical necrosis
- during obstetric emergency
- coagulative necrosis of glomeruli and tubules
mural thrombi embolizes
renal infarct - 25% CO
-white infarct- wedege shaped
sickle cell nephropathy
hematuria/hyposthenuria (can’t concentrate urine)
if biopsy kidney or do nephrectomy?
check for U/L kidney
hypoplasia
more commonly U/L
ectopic kidney
typically in pelvis
horseshoe kidney
fusion of lower poles
APKD gene
ADPKD
bilateral cysts
APKD always is bilateral
PKD1
ADPKD
- chromosme 16
- cell-cell matrix interactions
- worse prognosis - 70% ESRD by 60yo
- majority of cases
PKD2
ADPKD -chromosome 4 -Ca channel -better prognosis - 15% ESRD by 60yo -
ADPKD clinical
flank pain, stones, UTI, HTN
faster in blacks
also liver cysts, mitral valve prolapse, colon diverticula
berry aneurysms
increased in ADPKD
prognosis of ADPKD
40% die of coronary or HTN heart disease
perinatal ARPKD
90% cystic collecting ducts
-survive few hours after birth
MOST COMMON
neonatal ARPKD
60% cystic collecting ducts
-survive few months after birth
infantile ARPKD
20% cystic collecting ducts
-survive until childhood
juvenile ARPKD
10% cystic collecting ducts
-survive to adolescence
PKGD
fibrocystin - mutation in ARPKD
-chromosome 6
ARPKD
CD dilation
- cysts in cortex and medulla (cuboidal)
- dilated channels at right angles
also - portal fibrosis of liver occurs
cysts at corticomedullary junction
nephronophthisis and adult onset medullary cystic disease
most common genetic cause of ESRD in children and young adults
nephronophthisis
-polydipsia and polyuria
MCKD1 and 2
mutated in adult onset medullary cystic diseases
-autosomal dominant
mutations in nephronophthisis
NPHP1-11
JBTS2, 3, 9, 11
NPHP2
inversin
- modulates right/left patterning
- mutated in nephronophthisis
islands of undifferentiated mesenchyme, cartilage, immature CDs
multicystic renal dysplasia
U/L - excellent prognosis after removal of affected kidney
B/L - ESRD may occur
simple cysts
single/multiple
- typically in cortex
- incidental finding
- not clinically significant
proteus infection
magnesium ammonium phosphate
- struvite stones
- staghorn calculi
small stones
more hazardous - can enter ureter
subtotal intermittent obstruction
hydronephrosis
<1cm mass
renal papillary adenoma - benign
> 1cm mass
low-grade renal cell carcinoma
tuberous sclerosis
angiomyolypoma - benign
lesions of cerebral cortex, retardation, epilepsy, skin changes, heart tumors
TSC1 , 2
mutations in tuberous sclerosis
worry with angiomyolypoma
may hemorrhage significantly
epithelial cell with increased mitochondria
oncocyte
-in oncocytoma
oncocytoma
epithelial neoplasm
- large eosinophilic cells, from intercalated cells and CD
- mahogany with central scar
benign
wilms tumor
age 2-5yo
asian
large abdominal mass
pain, hematuria, HTN
WAGR
wilms tumor
aniridia
genital
retardation
denys drash
with wilms tumor
beckwith wiedeman
with wilms tumor
nephrogenic rests
precursor lesions in wilms tumor
diffuse anaplasia
bad prognosis for wilms tumor
older with wilms
better prognosis
p53, 11q, 16q deletion with wilms
bad prognosis