Renal: glomerular dzs, nephrotic, nephritic

Question 1

define glomerulopathies

Answer 1

significant cause of kidney disease

• acute and chronic
• group of diseases

Question 2

Glomerulonephopathies can result from

Answer 2

1. systemic inflammatory dz—>glomerulonephritis

2. non-inflammatory dzs–>metabolic dz–>DM, herediatary nephropathies like Alport’s

Question 3

primary glomerulopathies occur where

Answer 3

kidney

Question 4

secondary glomerulopathies occur

Answer 4

multisystem dz involving kidneys

–ex SLE

Question 5

degree of proteinuria determisn?

Answer 5

if its nephrotic or nephritic

Question 6

normal amt of protein in urine in 24 hrs?

Answer 6

100-200 mg/hr

Question 7

how does protein get into the urine?

Answer 7

• inflammation alters GBM (podocytes) permeability (normally resitricts things by size and charge…aka proteins)
• incrs in GBM permeability= pore sizes enlarge and RBCs and protein can pass through

Question 8

types of PU

Answer 8

1. orthostatic or postural–>elevated protein excretion while in the upright position and normal protein excretion in a supine or recumbent posiiton
2. overproduction
3. tubular
4. glomerular dysfunctino

Question 9

nephritis versus nephrotic

• define each
• basic CM for each one

Answer 9

Nephritic=caused by systemic inflammation/ immune complex dz (SLE, vasculitis) or an infection
*inflammation in glomerulus=BLEEDING
*renal vascular changes that cause HTN
*Proteinuria <3,000
TYPES=post infectious glomerulonephropathy (post strep), IGA nephropathy, Memabnoproliferative

Nephrotic= damange to filterting mechanism–leading to protein wasting in urine—>protein waisitng causes decr serum protein (hypoproteinemia.MC seen as hypoalbuminemia),
*decr serum protein= causes HYPOTONIC surroundings–>so fluid flows OUT of HYPOtonic–>

• EDEMA**
• *liver tries to compensate by producing more proteins…. and in doing so makes more lipids too
• Proteinuria >3,000
• hypoproteinuria
• hyperlipidemia

TYPES=minimal change dz, focal segmental glomerulura sclerosis, membranous nephropathy, DM nephropathy

Question 10

greater degree of proteinuria=?

Answer 10

nephrotic

>3,000

Question 11

injury to podocytes

Answer 11

nephrotic

Question 12

inflammation causing enalrged pores and “leaky” glomeruluar BM

Answer 12

nephritic

Question 13

describe the sediment for nephrotic

Answer 13

urine bands

urine >3,000 PU in 24/hrs

+lipidemia
+hyperlipidemia—>seen as oval fat bodies and maltese cross
+/- microscopic hematuria

Question 14

describe urine sed for nephritic

Answer 14

“acitve”

• hematuria
• RBC casts
• WBC casts
• Proteinuria variable… <3,000 BUT over >1 gram

Question 15

MC causes for transient PU

Answer 15

stessful conditions–>fever, exercise, CHF,

Question 16

major causes of nephritic syndrome

Answer 16

auto-immune or allergic interstitial inflammation

heavy metal intoxications

drug-induced intersitial injury

Question 17

major causes of nephrotic syndrome

Answer 17

primary and secondary glomerular dzs
dm
htn

Question 18

typical clinical findings in a pt with nephrotic syndrome

Answer 18

• **EDEMA (bc major loss of protein.. mainly albumin)
• hyperlipidemia
• hypoproteinemia-hypoalbuminemia

Question 19

define abnormal amt of PU

Answer 19

over 200 about

Question 20

Glomerulonephritis

-define

Answer 20

Inflammation/proliferation of glomerular tissue, scaring and fibrosis
-immune complexes (antigen/AB) form in ciruclation–>activation of compliment–>recruit and activate immune cells–>deposite in glomerulus–>cause damage to GBM, mesanial or capillary endothelium

ANTIGEN CAN BE TO:

• ->glomerular antigens
• ->glomerular BM
• ->trapped as circulating immune complexes
• ->cell mediated

**basically antigens/AB can be made directly against the GBM or there was an infection (strep) and those antigens/AB get trapped in glomerulus=inflammation

Question 21

mechanisms contributing to decline in GFR for glomerulonephritis

Answer 21

• ***decr glomerular perfusion secondary to
  1. inflammation
  2. scarring/sclerosis
  3. thickening of glomerular basememt membrane with increased permeability to proteins and RBC

Question 22

causes of glomerulonephritis

Answer 22

1. post infectious GN
2. IgA nephropathy
3. membranoproliferative glomerlunonephropathies (MPGN)
4. Rapidly progresive crescentic GNs

Question 23

MCC of nephritic syndrome

Answer 23

IGA nephropathy

Question 24

Post-infectious Glomerulonephritis

• mcc
• other causes

Answer 24

MC etiologies= STREP 2nd to nephritic strain of B-hemolytic GAS
other causes= SKIN or respiratory sources, Hep B, Hep C, Malaria, Syphilis, HIV

Question 25

IgA Nephropathy - another name - explain - MC population and gender - etiology

Answer 25

Berger Dz abnormal IgA immune complexes binds to glomerular mesangial cells-->cause injury *IGA is first line defense in respiratory and GI secretions---so infections cause an OVERPRODUCTION which then damage kidneys MC affects young males **24-48 HOURS after URI or GI infection

Question 26

Membranoproliferative GN - mcc - typical lab finding and what it means

Answer 26

immune complex mediated - involves mesanigal cell proliferation and complement deposition - gives glomurli a lobular apperance due to mesangial proliferation - the GBM splits--> causing Tram tracking or double counter apperance on histology ASSOC WITH HEP C---tx the hep c--- MPGN goes away

Question 27

HUS Vasculitis - often asoc with - explina patho

Answer 27

HEMOLYTIC UREMIC SYNDROME * *e. coli diarrhea shiga-toxin * MCC of ARF in kids * complement dysregulation via gene mutations * antibodies to complement factor H SECONDARY CAUSES -infection: shiga toxin producing E. coli or strep pneumoniae or HIV

Question 28

Lupus GN - mc type - pu levels

Answer 28

``` diffuse lupus nephritis class IV **alllllll over leads to progressive kidney dz ``` six types PU mostly nephritic--- but sometimes cna show nephrotic range

Question 29

rapidly progressive GN (RPGN) - characterized by? - types

Answer 29

* crescent lesions--acute chronic or mixed * cresents form from fibrin and plasma potein despotiion collapsing the cresecent shape of bowmans capsule * crescents may be secondary to severe injury to glomerular capillary wall - severity of dz correlates to degree of crescents * ***ANY CAUSE OF AGN CAN PRESENT WITH RPGN--the following ONLY PRESENT WITH RPGN TYPES 1. anti-GBM-->goodpastures 2. Pacui-immune--ANCA assoc vasculitis---> Wegners (granulomatosis polyangiitis and Microscopic polyangiitis

Question 30

Wegners aka Granulomatosis Polyangiitis - whart is it - (+) ??? - classically involves what part of body

Answer 30

systemic vasculitis of MEDIUM AND LARGE size arteries 90% are ANCA (+) ----> C-ANCA= 80%--> ANTI-PROTEINASE 3 ****** ----> P-ANCA=15% involves-->upper and lower resps, kidneys

Question 31

microscopic polyangiitis

Answer 31

renal small vessel vasculitis -usually renal limited *abesence of granulmatosis inflammation---how to tell the difference on biopy b.w this and wegners 70%= P-ANCA

Question 32

Goodpastures - type of rxn - explain age distribution

Answer 32

Type 2 hypersensitivity---AB mediated * ***ANTIBODIES TO: type IV collagen in lungs and kidney 1. anti-GBM---->GN (renal) 2. anti-alveolar BM--->alveolar hemorrhage (pulm) --->hemoptysis BIOMODAL age distribution---> 3rd and 6th decade of life (can presnt in 30s,,, and then again in 60s) M>>>F

Question 33

Nephrotic - cause thought to be secondary to? - primary etiologies - secondary etiologies

Answer 33

podocyte malfunction or injury PRIMARY ETIOLOGIES 1. membranous 2. Focal Segmental Glomerular Sclerosis 3. Minimal Change dz SECONDARY ETIOLOGY: 4. Diabetic Nephropathy

Question 34

reason for hyperlipidemia in nephrotic syndrome

Answer 34

increased synthesis from liver and decreased catabolism - hepatic lipoprotein synthsis is stimulated by decreased oncotic pressure-->hypercholesterolemia - also acquired defect in LDL receptor that causes diminished clearance - incrs trigs due to decreased catabolism NOT overproduction

Question 35

why are PT at risk for thrombosis in nephrotic syndrome

Answer 35

-DVT, PE, renal vein thrombosis -multifactorial etiology *increase urinary loss of anti-thrombin III, protein C and S *increased platelet aggregation BUT BOTH CAN LEAD TO ANASARCA----- total body swelling

Question 36

minimal change dz

Answer 36

-loss of negative charge of basement membrane promotes proteinuria **KIDS*** T-lymphocyte abnormalities that lead to increased glom. permeability---> effacement of visceral epithelial foot processes

Question 37

membranous nephropathy

Answer 37

increased glomerular permeability and glomerulosclerosis -idiopatic or -secondary forms *circulating IGG against antigen PLA2R on GBM

Question 38

Focal Segmental glomerulosclerosis (FSGS) | -

Answer 38

* idiopathic * primary * secondary * genetic 2nd to circulating factor toxic to the podocyte--can be adaptive "FOCAL" bc involves some glomeruli but not others "SEGMENTAL" bc involves a segment of glomerulus NOT THE WHOLE GLOMERULUS THO