Kidney Path

Question 1

_____ seen later in DN, caused by release of growth factors ___ and _____

high glucose and AGEs further stimulate this process

Answer 1

Tubulointerstitial fibrosis seen later in DN, caused by release of growth factors

ANG II and TGF B

Question 1

histology Thin BM disease (benign familial hematuria)

Answer 1

LM; normal

IF: negative

EM: thin GBM

Question 2

lipid guidelines for DN

Answer 2

LDL

Question 2

extra renal manifestations amyloidosis

Answer 2

Restrictive CM

macroglossia

splenomegaly

peripheral nerve

skin

joint

Question 3

reducing proteinuria via

Answer 3

Renin/ang/aldosterone blockage (renoprotective independent of BP)

Question 4

allport treatment

Answer 4

RAAS blockade

renal replacement necessary (2-3% will get anti-GMB disease)

Question 4

genetics thin basement membrane disease (Benign familial hematuira)

Answer 4

usually AD

Question 5

renal manifestations amyloidosis

Answer 5

kidney enlargment

HTN absent even with impaired renal fxn

proteinuria, albuminuria

Tubular defects: acidosis, polyuria, polydysia (from concenration defect)

Question 6

renal sequelae light chain deposits disease

Answer 6

proteinuria

hematuria

chronic renal insufficiency

Question 6

histology light chain deposition disease

Answer 6

LM: nodular glomerulosclerosis

IF: light chain kappa staining

EM: granular deposits along GBM

Question 7

stages of DN

Answer 7

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

Question 9

pathogenesis diabetic nephropathy hyperfiltration

Answer 9

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

Question 11

DN treatment

Answer 11

HTN therapy

glucose control

reduction of proteinuria

lipid lowering

lifestyle mod

Question 12

amyloidosis geneis in kidney

Answer 12

light chains secreted by single clone of B cells (usually from lambla light chains AL)

(systemic amyloidosis from chronic inflammation)

Question 13

hematuria pattern in allport syndrome

Answer 13

all males - microscopic hematuria

episodic gross hematuria precipitated URI

first 2 decades of life

90% of females have intermittent micro hematuria

Question 14

presentation Thin BM disease

Answer 14

continuous or intermittent microhematuria with or without gross hematuria

no renal insufficiency

(proteinuria, HTN, and ESRD rare)

Question 16

DN Complications

Answer 16

D Retinopathy

**Polyneuropathy **(Sensory polyneuropathy (D foot) Autonomic polyneuropathy (silent angina, gastropareis, ED, detrusor paresis)

**Miacrovascular complications **(stroke, CAD PAD)

Question 17

halmark mesangial changes of diabetic nephropathy

Answer 17

mesangial expansion

nodular diabetic glomerulosclerosis (acellular kimmelstiel-Wilson lesion)

Question 18

genetics alport syndrome

Answer 18

X recessive (or AD rec)

COL4A5 on Xq22 encoding a5 chain of IV collagen

> defect in BM

Question 19

widening of the GBM in diabetic nephropathy

Answer 19

accumulation of type IV collagen and reduction in negative charge heparin sulfate

podocyte widening processes, apoptopsis via Ang II and TGF B

> proteinuria

Question 20

extra renal manifestations Allport

Answer 20

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

Question 21

Renal manifestations Allport syndrome

Answer 21

Hematuria

Prtoeinuria develops later

HTN

ESRD (rate determined by mutation, sex differential)

Question 22

Rening/Ang/Aldosterone blockade therapuetic mechanism

Answer 22

blocking non-hemodynamic effects of AngII

blocking constriction in efferent artery > less glomeruli constriction and sclerosis

Question 23

pathogenesis diabetic nephropathy hypertrophy

Answer 23

increase in mesangial cells and capilary loops > increased filtration area > increased kidney size

Question 24

histology Allport

Answer 24

LM: normal early, segmental and global glomerulosclerosis, intersititial fibrosis

IF: negative or nonspecifc IgM, C

EM: thickening, thinning, basket weaving of GBM

Question 25

treatment thin BM disease

Answer 25

reassurance and follow up

BMP, urinalysis, blood pressure monitored every 2 years