Firecracker IV Flashcards
First presenting symptom of Goodpasture syndrome
hemoptysis
Second presenting symptom of Goodpasture syndrome
Hematuria
Why do you get hemoptysis (and cough and SOB) in Goodpasture syndrome?
Pulmonary hemorrhage
Why do you get hematuria in Goodpasture syndrome?
Rapidly progressive renal failure with S/Sx of nephritic syndrome
Three presenting signs of pulmonary hemorrhage
hemoptysis, cough, dyspnea
Presenting signs of RPGN
hematuria (2nd presenting symptom), RBC casts, azotemia, oliguria, mild/moderate hypertension, mild/moderate proteinuria and edema
Timeline of Goodpasure
Hemoptysis –> couple weeks –> hematuria
What are the deposits in IgA nephropathy
Mesangial deposition of IgA1, often with C3 and properdin
Which Ig is produced by mucosal tissue & follows infections of mucosal tissue
IgA
Adolescents/young adults with nephrotic syndrome and a nephritic component (eg, hematuria) → slow progression to renal failure
MPGN (membranoproliferative glomerulonephritis):
MPGN type I and type II glomerular appearance
Glomeruli are large and hypercellular due to WBC infiltrate, proliferation of mesangial cells and endothelial cells, and ↑ mesangial matrix
Mesangial ingrowth and new GBM (glomerular basement membrane) synthesis in response to deposition of immune complexes → duplication/splitting of GBM → thick GBM w/ “tram-track” or “double contour” appearance (esp. evident w/ silver or PAS stains)
MPGN types I and Ii
location of IC in MPGN type 1
Subendothelial
location of IC in MPGN type 2
membranoproliferative
complement in MPGN type 1
Activates both classical and alternative complement → ↓ serum C1, C4, C3
complement in MPGN type 2
- Activates alternative complement only → ↓ serum C3; normal C1 and C4
- Associated with C3 nephritic factor, an IgG autoantibody that stabilizes C3bBb (alternative complement C3 convertase) → persistent C3 activation → ↓ serum C3
composition of ICs in MPGN type 1
Subendothelial immune complex deposits of C3, IgG, and early complement components C1, C4
composition of ICs in MPGN type 2
Intramembranous immune complex deposits of C3 and IgG only
AI causes of secondary MPGN (type 1)
- SLE (systemic lupus erythematosus)
- SS (sjögren’s syndrome)
Infection causes of secondary MPGN type 1
- Hepatitis C, usually with cryoglobulinemia
- Hepatitis B
- HIV
- Schistosomiasis
malignancy causes of secondary MPGN type 1
- CLL (chronic lymphocytic leukemia)
- Lymphoma
besides panacinar emphysema, alpha1-antitrypsin deficiency causes
secondary MPGN type 1
benign congenital disorder characterized by cystic dilations of medullary collecting ducts (“swiss cheese” appearance of medulla on intravenous pyelogram); renal cortex is spared
Medullary sponge kidney
benign cysts usually located in the renal cortex of normal-sized kidneys.
Localized (simple) renal cysts
multiple cysts in cortex and medulla associated with end-stage renal disease and chronic dialysis therapy
Acquired renal cystic disease
Most patients are asymptomatic in medullary sponge kidney, but the most common symptoms/complications include:
1) Recurrent kidney stones (60% of cases)
2) Hematuria
3) UTI
Most common type of asymptomatic renal mass
simple renal cysts
most patients are asymptomatic with simple renal cysts, but what might still be found on urinanalysis
Microscopic hematuria is occasionally a benign, incidental finding on urinalysis.
Patients on dialysis with acquired renal cystic disease have an increased risk of
renal cell carcinoma
- Hereditary nephritis → hematuria, RBC casts
- Sensorineural hearing loss
- Ocular abnormalities
Alport syndrome
types of ocular abnormalities seen in alport syndrome
(e.g., lens dislocation, posterior cataracts, corneal dystrophy)
85% of cases of Alport syndrome are due to an X-linked recessive defect in the
α5 chain of type IV collagen
Regardless of inheritance pattern or the specific defect involved, all cases of Alport syndrome are caused by
defective type IV collagen assembly
defective type IV collagen assembly → structural-functional defects in the:
- GBM
- Cochlear hair cells of the inner ear
- Lens and cornea of the eye
Alport disease on EM
Irregular thickening/thinning of the GBM with splitting/lamination of the lamina densa → this often gives the GBM a unique “basketweave” appearance
Kimmelstiel-Wilson
diabetes nephropathy
nodular formation in diabetic nephropathy due to deposition of
type IV collagen (aka microangiopathy)
Common causes of pre-renal azotemia that can progress to ischemic ATN include
hypovolemia, impaired cardiac function, hypoalbuminemia, renal artery stenosis, NSAID use
ATN initiation phase is during the first 36 hours after the initial insult.
There is a slight decrease in urine output and an increase in BUN.
The maintenance phase starts 36 hours after the initial insult in ATN
marked oliguria and increasing BUN.
The recovery phase of ATN occurs when the tubule epithelium regenerates.
Diuresis is drastically increased, and BUN returns to baseline.
