Renal pathology I Flashcards
what are the congenital renal pathologies?
- agenesis
- hypoplasia
- ectopic
- horseshoe
is renal agenesis usually unilateral or bilateral?
bilateral
is renal hypoplasia usually unilateral or bilateral?
unilateral
horseshoe kidney carries an increased risk of ____________
infection
autosomal dominant polycystic kidney disease is due to mutation in what genes?
PKD1 or PKD2
1/3 of patients with AD polycystic kidney disease also present with cysts in what other organ?
liver
berry aneurysms are associated with what renal disease?
AD polycystic kidney disease (10% of patients)
PKD1 or PKD2 mutations cause what renal pathology?
AD polycystic kidney disease
how much of the kidney must be displaced with cysts before a precipitous drop in renal function?
2/3
further workup in ADPKD includes looking at what organs?
liver and brain (berry aneurysms)
what is the gross appearance of the kidney in ADPKD?
- no recognizable normal kidney tissue
- cysts containing hemorrhage causing a darkening appearance
does autosomal recessive polycystic kidney disease (ARPKD) present in childhood or adulthood?
childhood
ARPKD is due to a mutation in what gene?
PKHD 1
in ARPKD cysts develop from what renal structure?
collecting ducts
congenital hepatic fibrosis is associated with what renal pathology?
ARPKD
what is the gross appearance of the kidney at birth in ARPKD?
huge, white, SMOOTH surfaced kidneys
does ARPKD take on a more cystic or fibrotic morphology?
fibrotic
what are the other terms for simple cysts?
- “retention”
- “acquired”
renal biopsies are routinely stained for what factors?
- IgG
- IgA
- IgM
- C3
- C1q
- kappa light chains
- lambda light chains
anti-GBM disease has what type of immunofluorescence staining pattern?
linear capillary loop
- membranous nephropathy
- lupus nephritis
these have what type of immunofluorescence staining pattern?
granular capillary loop
- IgA nephropathy
- lupus nephritis
these have what type of immunofluorescence staining pattern?
mesangial
lupus nephritis can show what type of immunofluorescence staining patterns?
- granular capillary loop
- mesangial
what is the other name for the glomerular epithelial cell?
podocyte
in the pathogenesis of glomerular disease, antibodies are formed against what structures?
- native GBM
- “planted” antigens
- glomerular cells (mesangial cells, podocytes, etc)
what arm of immunity is associated with glomerular disease?
cell mediated
what cell types are associated with glomerular disease?
- neutrophils
- monocytes
- macrophages
- T cells
- NK cells
- platelets
- mesangial
what soluble mediators are associated with glomerular disease?
- cytokines
- chemokines
- coagulation factors
what are the three main clinical renal syndromes?
- nephritic
- nephrotic
- acute renal failure
what is the general cause of nephritic syndrome? what is the result?
- GLOMERULAR injury: breaks in glomerular capillary loops
- hematuria
what is the general cause of nephrotic syndrome? what is the result?
- GLOMERULAR injury: glomerular capillary filtration defects
- severe proteinuria
what is the general cause of acute renal failure? what is the result?
- tubular injury in most cases
- oliguria + rapid rise in serum creatinine
what is the first approach to diagnosis in a patient with renal disease?
classify the patient’s symptoms using clinical and laboratory parameters into one of the clinical renal syndromes
what are the symptoms of nephritic syndrome?
- HTN
- oliguria
- proteinuria
- azotemia
- hematuria
- RBC casts
what type of casts are seen in nephritic syndrome?
RBC casts
what are the symptoms of nephrotic syndromes?
- proteinuria (over 3.5 g / 24 h)
- pitting edema
- hypoalbuminemia
- hypercoagulable state
- hypercholesterolemia
- hypogammaglobinemia
- fatty casts
what type of casts are seen in nephrotic syndrome?
fatty casts
is azotemia seen in nephritic or nephrotic syndrome?
nephritic
what is the cause of hematuria in nephritic syndrome?
breaks in the glomerular capillaries that results in leakage of RBCs into urinary space
what is the cause of proteinuria in nephrotic syndrome?
glomerular capillary filtration defects (podocyte effacement) that results in leakage of protein into urinary space
what is the gross appearance of the kidneys in chronic renal failure?
- small kidneys
- thinning cortex
- increase in pelvic fat
what is the microscopic appearance of the kidneys in chronic renal failure?
- glomerular sclerosis
- interestitial fibrosis
- tubular atrophy
what are the three primary causes of nephrotic syndrome?
- minimal change disease
- focal segmental glomerulosclerosis
- membranous nephropathy
what is the main secondary cause of nephrotic syndrome?
diabetic nephropathy
- minimal change disease
- focal segmental glomerulosclerosis
- membranous nephropathy
these can cause what type of renal syndrome?
nephrotic syndrome
what is the most common cause of nephrotic syndrome in children (2-6 y)?
minimal change nephropathy (minimal change disease, lipoid nephrosis)
what is the histological appearance of minimal change nephropathy?
- diffuse epithelial foot process effacement
- lipid in tubular cells
minimal change nephropathy responds to what treatment?
steroids
“diffuse epithelial foot process effacement” describes the histological appearance of what disease?
minimal change nephropathy