Kidney Path Flashcards
_____ seen later in DN, caused by release of growth factors ___ and _____
high glucose and AGEs further stimulate this process
Tubulointerstitial fibrosis seen later in DN, caused by release of growth factors
ANG II and TGF B
histology Thin BM disease (benign familial hematuria)
LM; normal
IF: negative
EM: thin GBM
lipid guidelines for DN
LDL
extra renal manifestations amyloidosis
Restrictive CM
macroglossia
splenomegaly
peripheral nerve
skin
joint
reducing proteinuria via
Renin/ang/aldosterone blockage (renoprotective independent of BP)
allport treatment
RAAS blockade
renal replacement necessary (2-3% will get anti-GMB disease)
genetics thin basement membrane disease (Benign familial hematuira)
usually AD
renal manifestations amyloidosis
kidney enlargment
HTN absent even with impaired renal fxn
proteinuria, albuminuria
Tubular defects: acidosis, polyuria, polydysia (from concenration defect)
renal sequelae light chain deposits disease
proteinuria
hematuria
chronic renal insufficiency
histology light chain deposition disease
LM: nodular glomerulosclerosis
IF: light chain kappa staining
EM: granular deposits along GBM
stages of DN
1 Diabetes onset: glom hypertrophy, renal size, reversible transient albuminuria
**2 Asymptomatic **- mesangial expansion + GBM thickening
3 early nephropathy: HTN, microabuminuria, albumin secretion 30-300mg/day
**4. Overt Proteinuria: **urinary albumin >300mg/day, GFR decline, Retinopathy
**5. ESRD: **renal repalcement, 15 years after DM1 onset in patients with prot
pathogenesis diabetic nephropathy hyperfiltration
increased glucose dependent afferent arteiolar dilation >
>Angiotensin II constriction of efferent > hyperfiltration > increased colloid osmotic pressure in post glomerular cap > increased Na reab in proximal tubule > Angiotensin II hypetophy of proximal tubule
DN treatment
HTN therapy
glucose control
reduction of proteinuria
lipid lowering
lifestyle mod
amyloidosis geneis in kidney
light chains secreted by single clone of B cells (usually from lambla light chains AL)
(systemic amyloidosis from chronic inflammation)
hematuria pattern in allport syndrome
all males - microscopic hematuria
episodic gross hematuria precipitated URI
first 2 decades of life
90% of females have intermittent micro hematuria
presentation Thin BM disease
continuous or intermittent microhematuria with or without gross hematuria
no renal insufficiency
(proteinuria, HTN, and ESRD rare)
DN Complications
D Retinopathy
**Polyneuropathy **(Sensory polyneuropathy (D foot) Autonomic polyneuropathy (silent angina, gastropareis, ED, detrusor paresis)
**Miacrovascular complications **(stroke, CAD PAD)
halmark mesangial changes of diabetic nephropathy
mesangial expansion
nodular diabetic glomerulosclerosis (acellular kimmelstiel-Wilson lesion)
genetics alport syndrome
X recessive (or AD rec)
COL4A5 on Xq22 encoding a5 chain of IV collagen
> defect in BM
widening of the GBM in diabetic nephropathy
accumulation of type IV collagen and reduction in negative charge heparin sulfate
podocyte widening processes, apoptopsis via Ang II and TGF B
> proteinuria
extra renal manifestations Allport
Chochlear defects- adherence of organ of corti to basilar membrane
Ocular defects anteiror lenticonus, maculopathy, white/yellow flecks/grannualtions in perimacular distribution
Leiomyomatosis -esophagus and tacheobronchial tree
Renal manifestations Allport syndrome
Hematuria
Prtoeinuria develops later
HTN
ESRD (rate determined by mutation, sex differential)
Rening/Ang/Aldosterone blockade therapuetic mechanism
blocking non-hemodynamic effects of AngII
blocking constriction in efferent artery > less glomeruli constriction and sclerosis
pathogenesis diabetic nephropathy hypertrophy
increase in mesangial cells and capilary loops > increased filtration area > increased kidney size
histology Allport
LM: normal early, segmental and global glomerulosclerosis, intersititial fibrosis
IF: negative or nonspecifc IgM, C
EM: thickening, thinning, basket weaving of GBM
treatment thin BM disease
reassurance and follow up
BMP, urinalysis, blood pressure monitored every 2 years
