Thomas: Acute/subacute Renal Failure: Glomerular Disease
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Nephrotic Syndrome | Non-inflammatory
Damage to the basement membrane
Increased permeability
Proteinuria >3.5 g/d
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Increased premeability leads to... | ↓ albumin
↓ oncotic pressure
↓ effective circulating volume
Loss of Antithrombin III
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Nephritic Syndrome | Inflammatory
Immune-mediated damage
Infiltrates of neutrophils and phagocytes
-Crescentic lesions
-Rupture of GBM
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Clinical and lab findings with nephritic syndrome | Hematuria
Oliguria
HTN
RBC casts
Proteinuria <3.5g/d
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Glomerular diseases: Symptoms | Edema
Weight gain
Malaise
Oliguria
Hematuria
Worsening HTN
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Glomerular diseases: Past Medical Hx | HTN
DM
Hep B/C
HIV
Hyperlipidemia
DVT/PE
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Glomerular diseases: Family Hx | Kidney disease
DM
Autoimmune Dz
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Glomerular diseases: Physical exam | Edema, JVD, crackles
HTN
Conjunctival pallor
Signs of autoimmune dz
Xanthomas
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General labs/workup | Rule out pre/post-renal
Urine protein/Cr ratio
UA specifically for casts
Cholesterol panel
Albumin
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What tests are used to help rule out pre/post-renal ARF? | Bladder scan
UA
Specific gravity
FeNa
Uosm
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Nephrotic syndrome findings | Proteinuria >3.5
Serum albumin <3.5
Cholesterol >300 mg/dL
Oval fat bodies on UA
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Nephrotic syndrome findings (cont.) | ↓Ca due to ↓albumin
Anemia due to loss of transferrin and erythropoietin
Hypercoaguability due to loss of Antithrombin III
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Nephrotic Syndrome Tx | Statins
ACEI/ARBs
Fluid and salt retention
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Nephrotic Syndrome **BUZZWORDS** | Proteinuria
Hypoabluminemia
Edema
Hyperlipidemia
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Nephrotic syndromes | Minimal change disease
Focal segmental GS
Membranous GN
Diabetic GS
Amyloid
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Minimal Change Disease | Most common type in kids
90% of nephrotic cases <10
Sudden onset
Usually idiopathic
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Minimal Change Disease: Association and Damage | A:
Medications
Lymphomas
D:
effacement of podocytes
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Why do you get the nephrotic proteinuria with MCD? | Uniform thinning of GBM
Subepithelial accumulation of immune complexes
Disrupted filtration slits
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Minimal Change Disease Clinical and Dx | Edema
Proteinuria (up to 9g/d)
Dx: biopsy shows effacement of podocytes
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Minimal Change Disease Tx and prognosis | Tx:
High dose steroids for 8 weeks
Prognosis:
Very good, few progress to ESRD
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Focal Segmental Glomerulosclerosis (FSGS) | #1 cause of nephrotic syndrome in African Americans
~30% of adult nephrotic
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FSGS Mechanism | HTN causes hyperfiltration
Accumulation of ECM
Leads to scarring and tamponade
Epithelial cells damaged
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FSGS Associations | ***HIV***
IV heroin
Urethral reflux
Massive obstruction
Chronic pyelonephritis
Interstitial kidney dz
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FSGS Clinical | Usually asymptomatic
Microscopic hematuria initially
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FSGS Tx and prognosis | Tx
High dose steroids
Treat underlying cause
Prognosis
50% → ESRD in 5 yrs
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Membranous Glomerulonephropathy | From antibody/antigen deposition
Up to 1/3 adult nephrotic
2:1 M:F
Typical age 30-50
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Membranous Glomerulonephropathy Mechanism | Damage to mesangial matrix
Immune cells can't cross GBM so it's non-inflammatory
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Membranous Glomerulonephropathy Causes | Usually idiopathic
**Malignancies**
-Breast, colon, stomach, kidney, lung
Hepatitis B/C
Syphilis, DM, SLE
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Membranous Glomerulonephropathy Workup and Tx | Exclude SLE, RA
Hepatitis serologies
**age appropriate cancer screenings**
Tx:
Steroids and cytotoxic agents
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Diabetic Glomerulosclerosis Developes within... | 5-10yrs in DM II
10-20yrs in DM I
~50% diabetics
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Diabetic Glomerulosclerosis Increased prevalence in... | Smokers
Non-whites
Pts with poor glycemic and BP control
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Diabetic Glomerulosclerosis Dx | Urine microslbumin test
-microalb/Cr >30
Nodular sclerosis on biopsy
**Kimmelstiel-Wilson nodules**
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Diabetic Glomerulosclerosis Tx | Glycemic control
BP control with ACEI/ARBs
Intensive cholesterol control
(LDL<100)
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Nephritic disease | Inflammatory responce
Immune mediated damage
Crescentric lesions
Plug up Bowman's Capsule
Necrosis/thinning of GBM
Rupture of GBM
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Nephritic disease Clinical | ***********
*Hematuria*
*Oliguria *
* HTN *
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Nephritic disease Labs | UA:
Hematuria
RBC casts
Protein <3.5g/d
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Nephritic Diseases | IgA Nephropathy
Henoch Schonlein Purpura
Post-infectious GN
Membranoproliferative GN
Anti-GBM/Goodpasture's Syndrome
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What immunoglobulin plays a critical role in mucosal immunity? | IgA
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IgA Nephropathy | #1 cause of GN worldwide
2-3 days post infection
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Causes of IgA nephropathy | URT and GI infxn
Causes defective mucosal immunity
Overproduction and increased serum IgA
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How does a pt with IgA nephropathy present? | 1. Macroscopic or gross hematuria
2. Microscopic hematuria
3. High BP or kidney failure
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IgA nephropathy Clinical findings, Dx and Tx | Macroscopic hematuria
40% have asymptomatic microscopic hematuria
Normal complement (C3&4)
Tx
ACEI/ARBs, steroids
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Henoch-Schonlein Purpura | Disease of kids
Follows URI
IgA in:
-skin, GI tract, kidney, joints
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Henoch-Schonlein Purpura Clinical findings and Tx | Lower extremity bruising
Bloody diarrhea
Hematuria/HTN
Arthalgias
Tx: supportive
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Post-Infectious GN | Subendothelial antigen deposition
2-3 weeks post infxn
Low C3 and C4
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How is streptococcal pharyngitis diagnosed? | Rapid strep test
ASO titer
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Post-Infectious GN Dx and Tx | Dx:
Hx, ASO titer, anti-DNAase B
Tx:
none, self-resolving
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Membranoproliferative GN | Idiopathic cases uncommon
Usually secondary to other dz
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Membranoproliferative GN Associated with | Sjogren's Syndrome
SLE
Hep B/C WITH cryoglobulinemia
Infective endocarditis
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Membranoproliferative GN Labs and Tx | UA with dysmorphic RBCs, RBC casts, low C3 and normal C4
Treat underlying cause, steroids, antiplatelets
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Anti-GB/Goodpasture's Syndrome | 10-20% of cases of rapidly progressive GN
Can be ANCA+
Antibodies to type IV collagen
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Anti-GB/Goodpasture's Syndrome Clinical | Rapid onset of HTN
Oliguria
**Fatal if not treated promptly**
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Anti-GB/Goodpasture's Syndrome Kidney and lung involvement | Kidney only
More common in older women (Anti-GBM)
Kidney/lung
More common in young men (Goodpasture's syndrome)
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Goodpasture's Syndrome | 70% will have alveolar hemorrhage
(hemoptosis)
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Anti-GB/Goodpasture's Syndrome Dx and Tx | Dx
Anti-GBM antibodies, biopsy
Tx
Cyclophosphamide+steroids
Plasmapheresis if pulmonary hemorrhage
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Anti-GB/Goodpasture's Syndrome Prognosis depends on... | Promptness of Dx and Tx
Degree of kidney involvement
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Small Vessel Vasculitis | Wegener's Granulomatosis
-Lung, kidney, sinus
Microscopic Polyangiitis
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Small Vessel Vasculitis Clinical | Decreased appetite
Weight loss
Hematuria/proteinuria
Polymyalgia
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Small Vessel Vasculitis Dx and Tx | c-ANCA+
anti-PR3- Wegener's
anti-MPO- Microscopic
Cyclophosphamide+steroids
Plasmapheresis for Wegener's
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Nephrotic/Nephritic Lupus Nephritis | WHO classifications
III focal nephritis
IV diffuse nephritis
V Membrane formation
VI >90% of glomeruli sclerotic
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Nephrotic/Nephritic Lupus Nephritis Clinical and Dx | Acute onset of hematuria
Proteinuria
Edema
Pancytopenia
Dx: anti-Sm, anti-Ds DNA
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Nephrotic/Nephritic Lupus Nephritis Tx and prognosis | >Class III
Cyclophosphamide+/-steroids
Prognosis depends on
Kidney fxn
Histologic type
Crescents
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