Nephritic Syndromes Flashcards
What is the hallmark problem of nephritic syndrome?
Hematuria
What are potential origins of blood in the urine? What specialties would handle those?
Nephrologists deal with intrinsic hematuria (bleeding from glomeruli)
Urologists deal with extraglomerular hematuria
Nephritic Syndrome is characterized by…(list 3)
Hematuria with dysmorphic cells and RBC casts in urine
Some oliguria and azotemia
Hypertension
Why do RBCs become dysmorphic in nephritic syndrome?
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
What are RBC casts?
Groups of RBCs shaped like rectangles (casts)
They are molds of the renal tubules due to blood being filtered out at the glomerulus
What infection most commonly causes acute postinfectious glomerulonephritis?
Group A Beta hemolytic strep
When does acute postinfectious glomerulonephritis occur in relation to the original infection?
1-4 weeks after recovery (during the time when your body is mounting an antibody response to the infecting organism)
What are the typical symptoms of children with acute postinfectious glomerulonephritis?
Acute Nephritic Syndrome Hematuria Edema HTN Renal Failure
What is the microscopic problem causing acute postinfectious glomerulonephritis?
What might you see on electron microscope?
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)
What should you try to detect with immunofluorescence to confirm diagnosis of acute postinfectious glomerulonephritis?
IgG and C3 (complement)
What will lab tests reveal in acute postinfectious glomerulonephritis?
Tea-color urine (smoky, coca cola)
Hematuria, perhaps mild proteinuria
Elevated ASO titers
Low complement levels
What is the prognosis for children with acute postinfectious glomerulonephritis? Adults?
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)
What is the treatment for acute postinfectious glomerulonephritis?
Supportive care
What is the most common glomerular disease worldwide among kids and adults?
IgA nephropathy = Berger Disease
What is the basic clinical presentation of IgA nephropathy?
Recurrent gross and microscopic hematuria
When does IgA nephropathy present?
Typically see episodes of gross hematuria within 1-2 days of an upper respiratory tract, GI, or urinary tract infection
What is the pathogenesis of IgA nephropathy?
Mucosal infection leading to IgA production and deposition of IgA immune complexes in the mesangium
Where do immune complexes typically deposit in IgA nephropathy?
Mesangium
IgA immune complex deposits in the mesangium are AKA…
Electron-dense deposits
How is complement activation involved in IgA nephropathy?
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
What will lab tests typically reveal in IgA nephropathy?
Hematuria, perhaps with mild proteinuria
Normal complement levels
What is the prognosis for IgA nephropathy?
Variable.
Most are pretty benign, but may be prolonged and cause renal failure. Could be very aggressive in a small % of people.
What is the treatment for IgA nephropathy?
Supportive care
Hereditary nephritis commonly has a mutation in what protein?
Collagen Type IV
Where is collagen type IV important?
Glomerular basement membrane
Lens- eye
Cochlea - ear
What is the triad of symptoms seen in hereditary nephritis?
Nephritis
Nerve deafness
Various eye disorders, like early cataracts
How is hereditary nephritis typically inherited?
X linked inheritance, so mostly males have it
Describe the electron microscope diagnosis of hereditary nephritis
See a basket weave formation of the lamina densa
What is the prognosis for hereditary nephritis?
Overt renal failure between 20-50 years of age
What is the treatment for hereditary nephritis?
Supportive care
Kidney transplant
Counseling
Family testing
