Nephrotic Syndrome Flashcards by A B

define Nephrotic syndrome

Criteria need 3/4

lose over 3.5 g protein per day cannot be compensated by hepatic albumin synthesis –> decr plasma osmotic pressure –> edema

1) albuminuria
2) hypoalbuminemia
3) edema
4) hyperlipidemia

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Nephrotic syndrome due to …

incr permeability of glomerular capillary wall to plasma proteins

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incr permeability of capillary wall in Nephrotic syndrome due to either …

1) structural damage = decr size selectivity

2) loss of neg charge = decr charge selectivity

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causes of nephrotic syndrome include…

1) chronic glomerular disease

2) sclerosing glomerular disease

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major oncotic protein lost thru urine excretion is…

albumin and uncompensated by incr hepatic albumin synthesis

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what is effect of hypoalbuminmia

1) decr oncotic pressure
2) shift of fluid to extravascular space
3) edema
4) decr effective vascular volume –> Na+/H2O retention

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coupled to incr albumin synthesis is …

1) incr hepatic synthesis of LDL

2) hyperlipidemia or lipiduria

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clinical presentation of nephrotic syndrome (7)

1) edema
2) low serum albumin
3) 4+ proteinuria >3.5 g protein lost per hour
4) hyperlipidemia
5) lipiduria
6) malnourish in kids
7) lethargy

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complications of nephrotic syndrome

1) AT3
2) HDL
3) immunoglobulin/complement (factor B)
4) decr albumin
5) hypercholesterolemia
6) Na+/k+
7) vitamin d

1) incr coag factors, decr AT3, thromboembolic, renal VEIN thrombosis
2) accel atherogenesis
3) susceptibility to infection from encapsulated
4) edema, hypotension,
5) accelerated atheorgenesis
6) low Na, low K
7) decr vitamin d

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Non-inflamm causes of glomerular injury

circulating factors or Ig’s bind to GEC membranes or GBM without fixing complement –> lose charge selectivity first

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Inflamm causes of glomerular injury

1) complement fixing GEC Ab’s = alternative pathway
2) MAC incr permeability of GBM (lose size selectivity first)

–> membranous nephropathy

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Classic mechanism of edema formation in nephrotic

1) proteinuria
2) decr serum albumin
3) decr oncotic pressure
4) volume depletion
5) incr ADH/aldosterone
6) edema/Na+ retention

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More common mechanism (not classic) for edema in nephrotic syndrome

1) primary defect in Na+ excretion at collecting duct or

low serum albumin

2) edema (Na+ retention)
NO VOLUME DEPLETION JUST VOLUME EXPANSION
DECR ALDOSTERONE

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what does maltese cross indicate

lipid vacuoles free or in casts under polarized light

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biopsy everyone with nephrotic syndrome unless

1) diabetic

2) treat child first for minimal change disease

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if you see scarring in glomerulus, indicates …

if you see thicker membranes in glomerulus, indicates …

if you see hypercellularity in glomerulus, indicates…

1) focal sclerosis
2) membranous
3) proliferative

linear immunofluorescence indicates..

goodpasture’s

EM patterns include…

1) podocyte foot process fusion
2) subendo deposits = inner side of capillary wall
3) subepithelial (membranous deposits) = outside basement membrane

Hereditary nephrotic syndrome

1) etiology
2) similar to …
3) signs…
4) treatment …

1) mutation of slit diaphragm proteins - nephrin or podocin
2) minimal change or FSGS
3) ascites, edema, failure to thrive
4) resistant to steroids; transplant at 2 y/o

minimal change disease

1) presenting age
2) signs and symptoms
3) lab and urinalysis
4) assoc with …
5) due to?
6) treatment

1) 2-6 y/o
2) edema/ascites, HTN
3) normal or slight decr renal fxn
4+ protein, hyaline casts, microscopic hematuria RARE

4) allergy/atopy
Hodgkin’s lymphoma
NZSAIDS

5) T cell disorder

6) corticosteroids (prednisone)
or short course oral cytoxan for relapsers

Minimal change disease

1) on light microscopy
2) on EM
3) on IF

1) normal
2) negative
3) foot process fusion

effect of steroid therapy on urine protein in minimal change disease

patients respond very quickly to steroids but often relapses so need recurrent

or give oral cytoxan/cyclophosphamide

pathogenesis of minimal change disease

1) circulating factor
2) podocyte injury (fusion or alter charge density)
3) proteinuria

T cell hypothesis of minimal change

1) podocyte is epith cell but can become antigen presenting cell
2) podocyte turn on CD80 and secrete which can interact with CD4 T cell to release lymphokines and damage podocytes

FSGS | light microscopy

1) glomeruli normal appearing with focal segmental scarring in some glomeruli and within glomerulus

FSGS 1) age population/race 2) lab and urinalysis 3) assoc 4) pathophys 5) treatment 6) prognosis

1) 20-40 y/o african american 2) renal function normal or slight decr UA = 4+ protein, hyaline casts, MICROSCOPIC HEMATURIA COMMON 3) idiopathic or with HIV 4) circulating factor = suPAR viral factor = HIV infection of podocyte African american = APOL1 ``` 5) corticosteroids (pred) x 6 mo calcineurin inhib (cyclosporin) ``` 6) progress to dialysis but poor

etiology of focal sclerosis

1) idiopathic 2) IV heroin 3) HIV 4) sickle cell, obesity

Effect of suPAR in fSGS

1) suPAR expressed in podocytes 2) acftiv glomeruli and affect podocyte binding to basement membrane 3) proteinuria

APOL1 role in FSGS

tradeoff between APOL1 protective against sleeping sickness but leads to FSGS

HIV nephropathy 1) ___ syndrome 2) signs on microscopy 3) common populations 4) treatment

1) nephrotic 2) collapsing focal and segmental GN, reticuloendothelial inclusions, tubular dilation microcysts DUE TO VIRUS NOT HIV 3) 80% black, 50% IVDA, before AIDS 4) anti-retroviral

membrane nephropathy 1) etiology primary vs. secondary 2) presents with ___ syndrome 3) progress to ___ if untreat 4) age

1) primary = idiopathic 2/3 due to antibody against phsopholipase A2 on podocyte secondary = hep B, gold, mercury, SLE, cancer 2) nephrotic 3) CKD 4) 40-50 y/o

mechanism of injury in membrane neuropathy

1) antibody bind phsopholipase A2 on podocyte foot processes 2) damage podocyte directly 3) proteinuria

Screening for membranous nephropathy and carcinomas

1) H&P 2) CBC 3) CXR, mammogram 4) stool test for occult blood, sigmoidoscopy

HIV assoc membranous GN 1) found in ... 2) presents with 3) responds to..

1) chronic HBsAg carrier children 2) nephrotic syndrome 3) interferon

MPGN 1) presentation with ___ syndrome 2) lab tests 3) signs on microscopy 4) type 1 vs. type 2

1) nephrotic syndrome mildly decr renal function HTN 2) HCV positive, low serum complement 3) hypercellularity + GBM thickening, 4) type 1 = HCV, idiopathic, SLE type 2 = primary complement, post strep GN

Distinguish btwn type 1 and type 2 MPGN

both = mesangial prolif and GBM thickening type 1 = granular IgG subendo deposit type 2 = dense intramembranous deposit

how to test for type 1 vs. type 2

measure serum complement type 1 = low C3 and C4 type 2 = low C3 but normal C4

HCV assoc MPGN type 1 1) found in ... 2) labs 3) nephrotic or not 4) treatment 5) skin findings

1) adults with HCV 2) HTN elev creatinine low C3 and C4, 70% elev LFT 3) nephrotic or nonnephrotic proteinuria 4) inteferon 5) palpable purpura

palpable purpura indicates what GN

HCV assoc MPGN type 1