The nephrotic syndrome

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Presentation transcript:

The nephrotic syndrome Def: It is a clinico -biochemical state of many causes Features 1-Heavy proteinuria. 2-Hypoproteinemia.( decrease protein in the blood) 3-Generalized oedema. 4-Hyperlipidemia and lipiduria

Causes Systemic Diseases Renal 1-SLE 1-Membranous GN 2-DM 3-Amyloidosis 4-Infections e.g.; malaria, HBV,B Syphilis 6-lymphoma 7-Drugs:gold salt and NSAI Renal 1-Membranous GN 2-membranoproliferative GN 3-Minimal change GN 4-Focal segmental GS 5-Focal GN.(Mesangial,IgA Nephropathy)

Common features of nephrotic syndrome Gross -Enlarged pale kidney. -yellow ting due to fat resorption by tubular epithelium.

Microscopic 1-Glomeruli; LM&IF: Features specific to the disease. EM: Fusion of foot processes of podocytes. 2-Tubules 1-Hyaline droplets. 2-Vacuolar degeneration due to resorption of fat 3-Hyaline casts 3-Interstitial tissue variable oedema

Hyaline casts

Membranous GN Age: adults (30-50 ys) Patho: ICD Cause:- -Primary :Unknown (85%) -Secondary in course of infection like malaria syphilis, HBV, B, malignant tumors and gold salt therapy Patho: ICD

Microscopic Light: IF Thickening of the GBM Granular EM: flourescences to IgG and C3 EM: -Fusion of foot processes Of podocytes. -Subepithelial deposits

Thickened GBM IN MGN

Sikes formation in MGN. Silver stain

Spikes formation along GBM Silver stain

Diffuse granular fluorescence of GBM

EM in MGN, the darker electron dense immune deposits are seen scattered within the thickened basement membrane.

Membranous GN.EM Subepithelial Deposits

Prognosis: Remission and exacerbation, finally chronic renal failure. Clinical and laboratory findings Nephrotic syndrome Prognosis: Remission and exacerbation, finally chronic renal failure.

Minimal change GN Msc:1-Light Gross: as Nephrotic syndrome. Age: Commonest cause of nephrotic syndrome in children (1-4 ys ) Pathog: unknown or it is a disorder of T cells cytokines that cause loss of epithelial foot processes Gross: as Nephrotic syndrome. Msc:1-Light Glomeruli ; no changes.

2-IF: Negative. 3-EM: Fusion of foot processes of podocytes Tubules and interstitial tissues show changes of nephrotic syndrome 2-IF: Negative. 3-EM: Fusion of foot processes of podocytes Clinical and laboratory findings : as NS with selective proteinuria. Prognosis: good response to steroid therapy

Membranoproliferative GN Age; any age, mainly late childhood Pathogenesis; Type I; Common. It is ICD Type II (Dense deposit disease) :rare mediated by activation of the alternative complement pathway.

Accentuated lobulation Patchy irregular thickening of GBM MSC;1-Light Hypercellularity Double contour of GBM Accentuated lobulation Patchy irregular thickening of GBM

MPGN, the glomerulus has increased overall cellularity, mainly mesangial

MPGN.Lobulation. Patchy irregular Thickening of GBM

New matrix material is laid down resulting in replication of basement membrane material

Membranoproliferative GN type I

This silver stain demonstrates a double contour to many basement membranes, or the "tram-tracking" that is characteristic of MPGN

2-IF; Type I: Gr.fluor.to Igs and C3

Bright deposits in type II MPGN To C3

TYPE II, intramembranous deposit

Clinical and laboratory findings Presentation Asymptomatic proteinuria Nephrotic syndome+ hypertension Nephritic syndrome Prognosis : remission exacerbation and finally chronic renal failure

Focal segmental glomerulosclerosis Microscopic: -Sclerotic segments in some gl. and Hyalinosis -tubular atrophy -interstitial fibrosis IF: Granular fluorescence of the GBM for IgM andC3. EM:Fusion of foot processes and detachement of epithelial cells

Focal segmental GS

Clinically: Nephrotic syndrome, may be hypertension and microscopic hematuria. Prognosis: unfavorable (ending in chronic renal failure

Focal glomerulonephritis Cause: -idiopathic -In association of SBE,SLE, Henoch-schonlein PAN, and Goodpasture’s syndrome Pathogenesis: -ICD -Activation of the alternative complement pathway by aggregation of IgA.(Berger’s disease)

IgA Nephropathy (Berger’s Disease) -Common in children and young adults - Recurrent hematuria -It follows infection of the respiratory,GI and urinary tracts. -The IgA is deposited mainly in mesangium, which then increases mesangial cellularity

MSC: Focal and segmental proliferation of mesangial cells+ necrosis and crescent formation Clinically: Hematuria, proteinuria and may be nephrotic syndrome Course: Subsides without residual renal impairment

Focal glomerulonephritis IF:Granular

Focal GN Necrosis

Amyloidosis

Amyloidosis of the kidney

LM EM IF MPGN Minimal change Focal and Seg.GS Focal GN Lupus Nephritis Disease LM EM IF Membranous GN MPGN Minimal change Focal and Seg.GS Focal GN Lupus Nephritis

Chronic GN Grossly:-Small contracted kidney. Def: it is end stage renal glomerular disease. Grossly:-Small contracted kidney. -Granular outer surface. -Firmly adherent capsule. -Loss of differentiation bet. cortex and medulla. -Thick BVs at corticomedullary junction.

Chronic GN: Note contacted kidney& granular outer surface

Msc: Glomeruli: -Hyalinised and sclerotic. Tubules are atrophied and -Some are hypertrophied. Tubules are atrophied and dilated Interstitial fibrosis and chronic inflammatory cell infiltration Thick walle-blood vessels end arteritis obliterans

Chronic glomerulonephritis

Chronic GN Hyaline cast

Clinical and laboratory Findings: Prognosis: without Treatment is poor Urine changes -Polyuria. low Specific gra. -Mild albuminuria. -Hyaline and granular casts Marked hypertension Increase Bl. urea Prognosis: without Treatment is poor

Small- Sized Kidney (contracted kidney) 1-Hypoplastic kidney. 2-Chronic GN 3-Chronic PN 4-Senile(atherosclerotic) kidney. 5-Kidney of benign hypertension (Benign nephrosclerosis).

DM Effects of DM on the kidney: -Diabetic GS -Renal arteriolar sclerosis. -pyelonephritis. -papillary necrosis. Diabetic GS It leads to: a-Proteinuria. B-Nephrotic syndrome. C-CRF.

MSC: 1-Diffuse GS. -Diffuse increase in mesangial matrix -Thickening of GBM 2-Nodular GS. (kimmelsteil Wilson disease) Hyaline nodule is present in the mesangium, Containing fibrin and lipid. 3-Insudative lesion: -fibrin cap; eosinophilic focal Thickening of peripheral capillary loop. -Capsular drop: eosinophilic thickening of Bowman’s capsule

Diffuse glomeruosclerosis

Nodular GS

Nodular GS

Fibrin cap and Capsular drop

Lupus nephritis Classification; Presentation: Recurrent hematuria,nephritic s,nephrotic s,hypertension,CRF. Classification; -class I:Normal kidney. -Class II:Mesangial glomerular lesion. -Class III:Focal proliferaive GN. -Class IV:Diffuse Proliferative GN. -Class V:Membranous GN. -Class VI:Advancing sclerosing GN.

MSC of Class IV: Diffuse Proliferative GN -Diffuse hypercellularity due to Proliferation of endothelial cells and mesangial cells -Irregular thickening of GBM - Wire loop appearance -Few epith.crescents -Hematoxylin bodies.

Proliferative lupus nephritis Flea-Bitten appearance

Class II: Mesangial GN

Focal and segmental necrosis of glomerulus Class III: Focal GN Focal and segmental necrosis of glomerulus

Class IV:Diffuse Proliferaive GN Hematoxylin bodies Wire-Loop appearance

IF of Lupus Nephritis

EM of Lupus Nephritis

IF: Granular fluorescence of capillary walls for Igs and comploments EM: Subendothelial and mesangial electron dense deposits