Glomerular Dz

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Nephrotic Syndrome

par Annie John

Small intestine

par HH - 10MA 897880 Jean Augustine SS

Fuchs'Dystrophy

par Samia Tahir

Hyper and hypo glycemia

par Allison Ediger

Glomerular Dz

Nephrotic

Nephrotic syndrome

NephrOtic = PrOtein

FSGS

Reflux Neph: Unilateral renal agenesis

Obesity

Heroin

HIV

Rapid progression to ESRD w/in months

Collapsing features

Membranous (Other)

LN,membranous

(Neg) Congo Red

Fibrillary GN

Fibrils larger than amyloid

(Pos) Congo Red, Crystal Violet, Thioflavin T

Amyloidosis

Causes:

Eosinophilic, Glossy material

Microscopic

Homogenous, pink

Gross

Dark brown w/ Iodine

Renal transplant, Neg. for Immune complex Dz

Transplant GN

Most common cause of immune complex GN = Membranous GN

Must use IF and EM to rule out Membranous GN.

Subendothelial electron-lucent widening

White adult, Poor response to steroids

Membranous GN

White

Males > Females

RARE in children

ALL glomeruli involved

Compare microscopic findings to Acute-PIGN.

Stages (I-IV) determined by EM

If immune complex deposition is stopped at early stages, changes are reversible.

Numerous, small subepithelial deposits

Finely Granular

Opposed to Coarse granular (lumpy-bumpy) in PIGN

Diffuse, thickened BM

Black, ↓GFR, HTN, Poor response to steroids

Idiopathic FSGS

Prognosis: ESRD in 5-20 yrs

Epithelial cell detachment from BM

Focal damage of VECs

Visceral epithelial cells (Rapid Review)

Foam cells

Hyaline

Variant FSGS

Collapse of glomerular capillary loops

Obliterated capillary lumen

Segmental, Focal lesions

Juxtamedullary glomeruli

1-3 yo, T-cell cytokines affect BM charge, steroids

Min. Change Dz

Rx: Steroid therapy

Good prognosis (despite massive proteinuria)

Effacement of epithelial foot processes

May see mild increase in mesangial matrix

Nephritic

Think inflammation

NephrItic = Inflammation

anti-BM

GN+Lung, Type II Hypersens.

Male, Hemoptysis, Dyspnea, Discolored urine

Goodpasture's

5:1 M/F ratio

Pt develops renal failure typically?

GN, no lung

Vasculitis

HBsAg

Classic PAN

Renal infarct

P-ANCA

Hypersensitivity angiitis (microPAN)

Crescents possible

Focal necrotizing GN

Fibrinoid necrosis

Crescents

C-ANCA

Wegener's Granulomatosis

Lung Granuloma

Child: GI, Joint, Skin

Henoch-Schonlein purpura

Syndrome:

Abdominal Pain

Arthralgia

Purpura

Malar rash, Anti-Sm Ab

Few glomeruli, segment of glom. tuft

LN, focal prolif.

Females, 20s-40s

All Glomeruli involved

LN, diffuse prolif.

Most serious

Most common

Can also present w/

Female, 20s-40s

Subendothelial, Mesangial Deposits

"Full House"

"Full House" = Positive for the entire panel

Hyaline thrombi

Wire Loop

Normal OR

Hypocomplementemia

Both types are RESISTANT to STEROID THERAPY

Mesangial proliferation which advanced to the Basement membrane (HENCE MEMBRANOPROLIFERATIVE)

Alt. path of Complement

Low C3

Normal C4

MPGN-2 (DDD)

Entire BM is darkened

C3 Nephritic Factor

Mentioned in Intro. lecture as well

Immune Complex

Low C3

Low C4

MPGN-1

Subendothelial deposits

Split BM

IgG, C3

Duplicated BM

"Lumpy-bumpy", GAS

PIGN

Group A Strep (12, 4, 1, 49)

Latent period of 1-4 weeks

young>adult

male>female

Crescents possible

2 or 3 Large Humps

Coarsely granular = "lumpy bumpy"

Compare to membranous GN

Crescents may be seen

PMNs

Diffuse proliferative

Mild clinical abnormalities

Secondary

Benign HTN

Mild Diabetes

LN, mesangioprolif.

5 yr survival: 100%

Dense deposit in mesangium

IgG, Complement, IgA in Mesangium

Proliferation of mesangial cells, matrix

Primary

IgA Nephropathy

Not to be confused with Buerger's dz (thromboangiitis obliterans)

IgA Mesangial deposits

Before Dx, rule out the following

May see IgG and C3 also.

Mesangioprolif

Normal or...

Thin Membrane Dz

AKA Benign familial hematuria

AKA Benign idiopathic hematuria

Be cautious when making diagnosis.

There is no universally-accepted standard for normal GBM thickness

BM Thinning

XLR, Hearing, Ocular, a5 of Type IV collagen

Alport's

Rx: Renal transplant

EM

Multi-layer laminated

"basket-weave" appearance

IF

LM

↑ed mesangial matrix