Ch 20 Flashcards
analgesic nephropathy is associated w/
papillary necrosis
drug-induced hypersensitivity reaction of interstitiual and tubules leading to acute renal failure
tubulointerstitial nephritis
extensive accumulation of calcium phosphate crystals in tubules
acute phosphate nephropathy
HTN w/ esophageal varices in kids
ARPKD
pts w/ multiple myeloma associated with
light chain cast nephropathy (myeloma kidney)
northern european demographic
ADPKD
Sporadic: 80%
Hereditary:
- deletions on chromosome 3
- loss of VHL
clear cell carcinoma of kidney
polar scarring
chronic pyelonephritis w/ vesicoureteral reflux
fever, neurologic symptoms, microangiopathic hemolytic anemia
thrombocytic thrombocytopenic purpura (TTP)
corticomedullay cysts, shrunken kidneys, adults
adult-onset nephronophthisis
~ hematuria (most common)
- palpable mass
- flank pain
renal cell carcinoma
most common benign renal neoplasm
Renal Papillary Adenoma
most common cause of nephrotic syndrome in children
minimal change dz
irregular kidneys w/ cysts of various sizes, kidneys can be small, normal, or big
multicystic renal dysplasia
benign, small, discrete adenomas arising from the renal tubular epithelium
Renal Papillary Adenoma
salt-wasting, polyuria
adult-onset nephronophthisis
tram track apperance
MPGN I
worse pronosis of SLE nephropathy
class 4: diffuse lupus nephritis
kimmelstiel-wilson nodules on histo
diabetic nephropathy
liver cysts
ADPKD
cortical surface of kidneys w/ multiple foci of yellow grey areas of acute inflammation and abscesses
acute pyelonephritis
vesicoureteral reflux is a predisposing factor for
pyelonephritis
PKHD1 on chromosome 6 encoding fibrocystin
ARPKD
most common adult kidney cancer
Renal Cell Carcinoma
bence jones proteins
light chain cast nephropathy (myeloma kidney)
gross kidney: granular, pitted surface, thin cortex, cortical depressions
diabetic nephropathy
defect in alpha 5 chain of type 4 collagen
x-linked
alport syndrome
pale cortex
diffuse cortical necrosis
hematuria
polyruia
HTN
ADPKD
reduced calyces
chronic pyelonephritis
collapsing glomerulosclerosis in HIV patients
FSGS
inherited mutations of complement regulatory proteins like factor H
Atypical hemolytic uremia syndrome (HUS)
multifocal ischemia of kidney and parenchyma
benign nephrosclerosis
nephrotic dz might be associated w/ hodgkin lymphoma
minimal change dz
crescents composed of fibrin and macropahges
RPGN
- Wilms tumor
- Neonatal hypoglycemia
- muscular hemihypertrophy
- organomegaly (ex: tongue)
beckwith weidmann syndrome
reduced sized kidneys, ischemic atrophy, mild arteriosclerosis
renal artery stenosis
most common genetic cause of end-stage renal dz in children and young adults
familial juvenile nephronophthisis
nodular glomerulosclerosis
diabetic nephropathy
ethylene glycol causing oxalate crystal is associated w/
acute tubular injury
thick glomerular membranes, subepithelial deposits, granular deposition on IF, spikes
membranous nephropathy
progressive sclerosis that develops after many types of renal injury and leads to proteinuria
FSGS
less than 1cm in diameter
- always in the cortex
- pale yellow-grey, discrete, well circumscribed nodules
Renal Papillary Adenoma
eating e.coli
typical hemolytic uremia syndrome (HUS)
endothelial injury causing thrombocytopenia leading to microangiopathic hemolytic anemia
typical hemolytic uremia syndrome (HUS)
- gonadal and renal tumors
denys drash syndrome
medullary cysts, shrunken kidneys, cortical tubulointerstitial damage
medullary sponge kidney
hematuria, hyposthenuria, patchy papillary necrosis, proteinuria
sickle cell nephropathy
renal artery stenosis in older patients associated w/
atheromatous plaque
IgA and C3 deposits in the mesangium
henoch-schonlein purpura
tumor comprised of blastema, primitive glomeruli and tubules, and stromal cells (triphasic)
wilm’s tumor
hepatic fibrosis
ARPKD
IgG, IgM and C3 on IF
subendothelial deposits on EM
MPGN I
subendothelial and mesangial dense deposits w/ wire loops on LM
SLE nephropathy
ADAMTS13 mutation
thrombocytic thrombocytopenic purpura (TTP)
glomerulus “stuffed” in bowman’s capsule w/ decrease in urinary space
SLE nephropathy
corticomedullay cysts, shrunken kidneys, children
familial juvenile nephronophthisis
flea bitten appearance of kidney
malignant nephrosclerosis
- Wilms tumor
- Aniridia
- Genital abnormalities
- Mental and motor Retardation
WAGR syndrome
50% of pts have concomitant bladder tumors
Renal Urothelial (transitional cell) Carcinoma
can spontaneously rupture with massive hemorrhage –> initial presentation may be shock secondary to massive retroperitoneal and/or intra-abdominal hemorrahge
angiomyolipoma
nephrotic syndrome associated with hep B, C, SLE, NSAIDs
membranous nephropathy
hyperplastic arteriosclerosis
malignant nephrosclerosis
EM: packed with mitochondria
oncocytoma
most common cause of glomerulonephritis
IgA nephropathy
increased in frequency in pts with tubular sclerosis
angiomyolipoma
associated w/ gluten enteropathy
IgA nephropathy
IgM and C3
effacement of foot processes
FSGS
mutations in vWF
thrombocytic thrombocytopenic purpura (TTP)
hyaline arteriosclerosis
benign nephrosclerosis
mahogany-brown and well circumscribed often with central stellate scar
oncocytoma
radial arrayed cysts
ARPKD
dirty brown granular casts
acute tubular injury
arise from type A intercalated cells of renal cortical collecting ducts
oncocytoma
increased mesangial matrix w/ alterations in GBM
MPGN I
amyloidosis, hypercalcemia, hyperuricemia
light chain cast nephropathy (myeloma kidney)
tx PSGN
supportive
most common cause of nephrotic syndrome in caucasian adults
membranous nephropathy
formation of uric acid crystal in renal tubules in collecting ducts
acute urate nephropathy
thinning and thickening of bm
alport syndrome
originates from utothelium of renal pelvis
infiltration of wall of the pelvis and calyces is common
Renal Urothelial (transitional cell) Carcinoma
WT1 chromosome 11 mutations
WAGR and denys drash
AR inherited mutation of NPHP1
familial juvenile nephronophthisis
- proteinuria
- hypoalbuminemia
- hypogammaglobunemia
- hypercoaguable state
- hyperlipidemia
nephrotic syndrome
embolization of atheromatous plaques from aorta or renal artery into intrarenal vessels
atheroembolic renal dz
inherited form associated w/ mutations in nephrin, NPSH1, podocin, alpha-actinin 4, TRPC6, APOL1
FSGS
hypercellularity of glomerulus
nephritic syndrome
thickened GBM and increased mesangial matrix w/ shrunken kidneys
diabetic nephropathy
most common cause end stage renal dz
diabetic nephropathy
AR mutations in alpha3 or alpha4 of type 4 collagen
thin basement membrane dz
AD inheritance
- associated with loss of TSC1 or TSC2
angiomyolipoma
“gouty nephropathy”
chronic urate nephropathy
purpuric skin lesions, abd pain, vomiting, arthralgias
henoch-schonlein purpura
permeation of lamina densa of GBM by electron dense ribbon of materia
MPGN II
berry aneurysm
MVP
diverticular dz
ADPKD
demographics for benign nephrosclerosis
older pts
african americans
demographics malignant nephrosclerosis
younger patients
men>women
african americans
C3 nephritic factor autoantibodies
MPGN II
lipid deposition in proximal tubule
minimal change dz
oliguria, fever, rash, azotemia after starting a drug
tubulointerstitial nephritis
hamartoma comprised of blood vessels, smooth muscle, and adipose tissue
angiomyolipoma
occurs after obstetric emergency
diffuse cortical necrosis
hemorrhage, erythrocytosis, neoplasia
shrunken kidneys
acquired renal cystic dz
“white” wedge shapes
renal infarcts
cholesterol cysts
atheroembolic renal dz
tx is inhibitors of RAAS syndrome
FSGS
Sporadic: - trisomy 7, 16, 17 - lost Y (MET proto-oncogene) Hereditary: - trisomy 7
papillary carcinoma of kidney
renal artery stenosis in young women associated w/
fibromuscular dysplasia
anti-PLA2R antibodies
membranous nephropathy
yellow-green to pink tubular bile casts, pts w/ liver dz
bile cast nephropathy
hepatic cysts
ADPKD
genetic defects causing ADPKD
PKD1 on chromosome 16: polycystin 1
PKD2 on chromosome 4: polycystin 2
risk factors:
- antiphospholipid antibody syndrome
- pregnancy
- oral contraceptive
- scleroderma
- chemotherapy
Atypical hemolytic uremia syndrome (HUS)
recurrent hematuria
IgA nephropathy
diarrhea, hematemesis, melena, oliguria, hematuria
typical hemolytic uremia syndrome (HUS)
diffuse thinning of GBM
thin basement membrane dz
second most common cause of acute kidney injury
drug induced interstitial nephritis
hepatic fibrosis w/ hepatic failure and HTN in kids
ARPKD
C3 deposits in basement membrane in circular aggregation w/o IgG
MPGN II