nephrotic and nephritic syndrome (glomerulonephritis)

Question 1

nephrotic syndrome

Answer 1

proteinuria >3.5 g/day/1.73 m2
hypoalbuminemia <3.5 g/dL
edema
hyperlipidemia
lipiduria

Question 2

causes of primary idiopathic NS

Answer 2

minimal change disease, membranous nephropathy, focal segmental glomerulosclerosis, membranoproliferative glomerulonephritis (overlap)

Question 3

causes of secondary NS

Answer 3

minimal change disease, membranous nephropathy, focal segmental glomerulosclerosis, membranoproliferative GN, diabetic nephropathy, amyloid, light change deposition disease

Question 4

minimal change disease

Answer 4

most cases primary/idiopathic. secondary causes: NSAIDS, malignancies (hematologic)

Question 5

focal segmental GN

Answer 5

secondary causes: healing of previous glomerular injury, massive obesity, OSA, sickle cell anemia, HIV, pamidronate, heroin abuse

Question 6

membranous nephropathy

Answer 6

secondary causes: malignancy, primarily solid tumors, class V lupus nephritis, rheumatoid arthritis, hepatitis B and C, drugs, syphilis

Question 7

definition of GN

Answer 7

intraglomerular inflammation, cellular proliferation, hematuria, excludes nonproliferative disorders

Question 8

presentation of GN

Answer 8

varies from microscopic asymptomatic hematuria or proetinuria to acute nephritis, to rapidly progressive nephritis.

Question 9

nephritic syndrome

Answer 9

hematuria-dysmorphic red blood cells, RBC casts
azotemia
oliguria
hypertension
variable proteinuria

Question 10

focal proliferative GN

Answer 10

IGA nephropathy
henoch-schonlein purpura
lupus nephritis (class 1 and 2)
heriditary nephritis (alport’s)

Question 11

diffuse proliferative GN

Answer 11

poststrep GN
bacterial endocarditis
lupus nephritis (class IV)
membranoproliferative gn
crescentic gn
vasculitis

Question 12

IGA nephropathy

Answer 12

common cause of GN
mesangioproliferative GN
asians and caucasians
rare in african americans
age 20-30 males>females
pathogenesis-altered regulation of IGA

Question 13

clinical presentation of IGA nephropathy

Answer 13

episodic gross hematuria
persistent microscopic hematuria
acute GN
ESRD

Question 14

treatment

Answer 14

no cure
N-3 fatty acids (fish oil)
corticosteroids
ace/arbs

Question 15

henoch-schonlein purpura

Answer 15

systemic IgA nephropathy

• arthralgias
• purpura
• ab pain
• GI bleeding
• hematuria

Question 16

clinical presentation of poststrep GN

Answer 16

children 2-10 years
uncommon over age 40
symptoms develop 7 days to 12 weeks after the infection 
low complement levels
spontaneous recovery is the rule
hematuria can persist 6 months
proteinuria, mild can persist years

Question 17

pathogenesis

Answer 17

nephritogenic strains of streptococci
planted antigen
-nephritis associated plasmin receptor GAPDH
-zymogen
host immune response
alternative pathway of complement activation
IgG and C3 found in glomeruli

Question 18

rapidly progressive GN

Answer 18

GN (nephritic syndrome)
rapid decline in renal funcion 
rare-2-4% of all GN
pathologic hallmark crescents
calssified based on presence or absence of immune complexes

Question 19

anti GBM disease presentation

Answer 19

bimodal age distribution (3rd and 6th decades)
pulmonary hemorrhage
malaise, fatigue, anorexia, weight loss, arthralgias, myalgias
caucasians
rare in african americans

Question 20

pathogenesis of anti GBM disease

Answer 20

antibodies develop against alpha 3 chain type IV collagen in GBM. linear deposition of IgG along GBM. antibodies detected by ELISA. ANCA found in about 30% pts

Question 21

treatment of anti GBM disease

Answer 21

outcome poor without therapy. corticosteroids alone insufficient. cyclophosphamide, plasma exchange with albumin 14 days. renal recovery rare if pts present needing dialysis