Basic presentations
Heavy proteinuria (>3.5gm/day)
Hypoalbuminemia
Edema
Hyperlipidemia/Lipiduria
Normal Complement levels
Diabetic Nerophaty
Info
Leading cause of ESRD (1/3 of all patients)
Seen in 25-40% of type 1/2 diabetics
Pathogensis
Systemic hyperglycemia
Reccurance after transplant
Glomerulosclerosis
Earliest lesions
Hyperfiltartion injury
Mesangium epxansion
Thickening of GBM
Later Lesions
Diffuse global glomerulosclerosis
More of Early lesions
Kimmeslstiel-Wilson nodules
Lipids/Fibrin
Fibrin Cap/Capsular drop
Plasma proteins
Ischemia
Tubular atrophy/interstitial fibrosis
Hyaline arteriolosclerosis
Timeline
Initially
Increased GFR/ Glomerular hydrostatic pressure
7-13 yrs
Microalbuminuria
.3- 3g/24 hr
10-20 yrs
Macroalbuminuria
Nephrotic syndrome
20+ yrs
Persistent heavy proteinuria
HTN
Decline in GFR 1-24
Median 12
Minimal Change
Info
Children/ 15% in adults
Idiopathic
Can also be seen
Lymphoma
Renal cell carcinoma
Labs
Serum
Low albumin
Selective protienuria
Normal creatinine
Urine
Bland Urine sediment
Physical Exam
No HTN
Edema
Periorbital
Pedal
Progression
Remission with Steroids within 8 weeks
1/3 Rule
No Relaspe
Few Relaspes
Many Relaspes
Not likely to progress to CRF/ESRD
Adults have worse prognosis
Pathogensis
Podocyte
diffuse effacement and detachement
No immune complex/inflammatory injury
May involve circulating glomerular permeability factors
Pathology
LM
N
IF
N
EM
Fusion of foot processes
FSGS
Info
10-15% of idiopathetic nephrotic syndromes seen in children
Higher incidence seen in adult AA
Secondary to
HIV
Morbid obesity
Chronic reflux nephropathy
Heroin
Malignancies(lymphoma)
Progression
Present asymptomatic proteinuria
Progresses to Massive Proteinura/Microscopic hematuria
HTN
Persistent proteinuria/progressive decline in renal function
ESRD by 5-20 yrs
50% recurrence post transplant
Secondary FSGS
Anything that causes a reduction in renal mass
Hyperfiltration Injury
Pathology
LM
FSGS
IF
Negative or Non Specific granular IgM/C3 deposits
EM
Patchy fusion of foot processes/effacement
Membranous Nephropathy
Info
Presentation
Nephrotic syndrome
Microscopic hematuria (50%)
HTN
Late Renal Insufficiency
20 year follow up
25% spontaneous remission
50% persistent proteinuria/ stable or gradual loss of renal function
25% develop ESRD
Renal Vein Thrombosis
Progression
Bad Progonsis
Males
>50
>10gm proteinuria
Pathogenesis
IC deposition
Sub Epithelium
In situ/ cirulating
Heymann Nephritis
Supports in situ
Complement Activation
MAC activated damage of GFM
Associated Causes
DNA SLE
Tumors
Hep B
Syphilis
Malaria
Captorpril
Mercury
Gold
Penicillamine
Pathology
LM
Diffuse thickening of GBM w/ minor increase in cellularity
IF
Subepithelial fine granular deposits of IgG/C3 in BM
EM
Subepithelial IC deposits with GBM spike formation
Amyloidosis
Pathology
LM
Nodular, amorphous hyaline matierial in mesangium/capillary lopps
Congo Red Stain
(+) w/ apple green birefringence
EM
Subendothelial/Mesangial fibrils
Presentation
Either caused by AL or AA amyloids
Nephrotic syndrome
Renal insufficiency in 50% by diagnosis
Electrolyte abnormalities
Fanconi's syndrome (loss of renal tubal reabsorptive function)
Other Systemic Presentations
Heart
CHF
Arrhythmias
Heart Block
GI
Hepatomegaly
Malabsorption
Hemarrahge
Neuro
Ischemic stroke
Neuropahty
Orthostatic hypotension
Skin
Easy bruising
Purpura
Diagnosis
Biopsy needed
Very poor prognosis
Survival drops severely as yrs pass 50% yr 1 to 5% 10 years
Death due to end-organ failure of affected organ
Secondary Amyloid (AA caused)
RA
Behcet syndrome (systemic inflammation of blood vessels)
Chrons
Osteomyelitis
Renal cell carcionma
Hodgkins lymp