Nephritic Syndromes

Question 1

What is the hallmark problem of nephritic syndrome?

Answer 1

Hematuria

Question 2

What are potential origins of blood in the urine? What specialties would handle those?

Answer 2

Nephrologists deal with intrinsic hematuria (bleeding from glomeruli)

Urologists deal with extraglomerular hematuria

Question 3

Nephritic Syndrome is characterized by…(list 3)

Answer 3

Hematuria with dysmorphic cells and RBC casts in urine

Some oliguria and azotemia

Hypertension

Question 4

Why do RBCs become dysmorphic in nephritic syndrome?

Answer 4

You may see both normal and deformed RBCs in urine. The RBCs travel through punctures in the glomerular membrane and they may become pushed and squeezed forcefully to make their way out

Question 5

What are RBC casts?

Answer 5

Groups of RBCs shaped like rectangles (casts)

They are molds of the renal tubules due to blood being filtered out at the glomerulus

Question 6

What infection most commonly causes acute postinfectious glomerulonephritis?

Answer 6

Group A Beta hemolytic strep

Question 7

When does acute postinfectious glomerulonephritis occur in relation to the original infection?

Answer 7

1-4 weeks after recovery (during the time when your body is mounting an antibody response to the infecting organism)

Question 8

What are the typical symptoms of children with acute postinfectious glomerulonephritis?

Answer 8

Acute Nephritic Syndrome
Hematuria
Edema
HTN
Renal Failure

Question 9

What is the microscopic problem causing acute postinfectious glomerulonephritis?
What might you see on electron microscope?

Answer 9

Deposition of immune complexes (with IgG) on the glomerular capillary wall, leading to leukocyte and PMN infiltration and damage to the structural integrity of the wall.

There is also in situ immune complex formation, forming big subendothelial deposits (humps)

Question 10

What should you try to detect with immunofluorescence to confirm diagnosis of acute postinfectious glomerulonephritis?

Answer 10

IgG and C3 (complement)

Question 11

What will lab tests reveal in acute postinfectious glomerulonephritis?

Answer 11

Tea-color urine (smoky, coca cola)
Hematuria, perhaps mild proteinuria

Elevated ASO titers

Low complement levels

Question 12

What is the prognosis for children with acute postinfectious glomerulonephritis? Adults?

Answer 12

95% of kids will recover fully

Adults have more atypical presentations and less favorable outcomes (may progress to end stage kidney disease or rapidly progressing renal failure)

Question 13

What is the treatment for acute postinfectious glomerulonephritis?

Answer 13

Supportive care

Question 14

What is the most common glomerular disease worldwide among kids and adults?

Answer 14

IgA nephropathy = Berger Disease

Question 15

What is the basic clinical presentation of IgA nephropathy?

Answer 15

Recurrent gross and microscopic hematuria

Question 16

When does IgA nephropathy present?

Answer 16

Typically see episodes of gross hematuria within 1-2 days of an upper respiratory tract, GI, or urinary tract infection

Question 17

What is the pathogenesis of IgA nephropathy?

Answer 17

Mucosal infection leading to IgA production and deposition of IgA immune complexes in the mesangium

Question 18

Where do immune complexes typically deposit in IgA nephropathy?

Answer 18

Mesangium

Question 19

IgA immune complex deposits in the mesangium are AKA…

Answer 19

Electron-dense deposits

Question 20

How is complement activation involved in IgA nephropathy?

Answer 20

IgA is a poor activator of the complement system. Most activation in IgA nephropathy occurs through the alternative pathway.

Complement levels are usually not decreased in IgA nephropathy

Question 21

What will lab tests typically reveal in IgA nephropathy?

Answer 21

Hematuria, perhaps with mild proteinuria

Normal complement levels

Question 22

What is the prognosis for IgA nephropathy?

Answer 22

Variable.

Most are pretty benign, but may be prolonged and cause renal failure. Could be very aggressive in a small % of people.

Question 23

What is the treatment for IgA nephropathy?

Answer 23

Supportive care

Question 24

Hereditary nephritis commonly has a mutation in what protein?

Answer 24

Collagen Type IV

Question 25

Where is collagen type IV important?

Answer 25

Glomerular basement membrane

Lens- eye

Cochlea - ear

Question 26

What is the triad of symptoms seen in hereditary nephritis?

Answer 26

Nephritis
Nerve deafness
Various eye disorders, like early cataracts

Question 27

How is hereditary nephritis typically inherited?

Answer 27

X linked inheritance, so mostly males have it

Question 28

Describe the electron microscope diagnosis of hereditary nephritis

Answer 28

See a basket weave formation of the lamina densa

Question 29

What is the prognosis for hereditary nephritis?

Answer 29

Overt renal failure between 20-50 years of age

Question 30

What is the treatment for hereditary nephritis?

Answer 30

Supportive care
Kidney transplant
Counseling
Family testing