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Nephrotic Syndrome
Al Buick
Nephrotic Syndrome Definition Structure of glomerulus Causes
Minimal change Membranous Glomerulonephritis Focal segmental Glomerulosclerosis
Investigations Prognosis and complications Treatment Random quiz
Definition
Manifestation of glomerular disease defined by: Proteinuria (>3.5g per day) Hypoalbuminaemia (< 30 g/l) Generalized oedema and
hyperlipidaemia
Structure of GlomerulusFenestrated endothelial
cellsGBM
Podocytes found on
Glomerular epithelial cells
Zip like structure including from proteins e.g. nephrinLinker proteins include podocin
1. GBM – certain collagens and heparin like molecules arranged. Size and charge selection. Albumin not pass, haemaglobin can (67,000) Myoglobin (17,000) and monomeric light chains (22,000) can pass through
2. Between podocytes a thin digapragms consisting of proteins such as nephrin(mutations of proteins such as nephrin have been found to cause some nephritic syndrome)
3. Normal urine contains small amount of protein (150mg/day)
Causes Primary glumerular disease
Minimal change Membranous glomerulonephritis Focal segmental glomerulosclerosis
Systemic Disease Diabetes SLE Amyloid
Drugs NSAIDS Penicillamine Gold
• Neoplasm• Any sold organ tumour• Leukaemia• Lymphoma
• Infection• Malaria• Streptococcal• Hep B and C• HIV
• Vascular• Malignant hypertension
CausesHistological classification
Nephrotic Syndrome
Minimal Change Disease
Focal segmental Glomerulosclero
sis
Membranous Glomerulonephriti
s
CausesHistological classification
Nephrotic Syndrome
Minimal Change Disease
Focal segmental Glomerulosclero
sis
Membranous Glomerulonephriti
s
Minimal Change
Minimal Change
Commonest cause in children 80 – 90% GN in children (20% in adults) More common in boys
Only 1% lead to chronic renal failure 80% have recurrence Responds well to treatment Associations e.g. Hodgkins
lymphoma
CausesHistological classification
Nephrotic Syndrome
Minimal Change Disease
Focal segmental Glomerulosclero
sis
Membranous Glomerulonephriti
s
CausesHistological classification
Nephrotic Syndrome
Minimal Change Disease
Focal segmental Glomerulosclero
sis
Membranous Glomerulonephriti
s
Membranous Glomerulonephritis
Membranous Glomerulonephritis
20 – 30% of nephrotic syndrome in adults (2-5%children)
Primary or idiopathic but can be secondary such as Automimmune – SLE, thyroid disease Drugs – gold, penicillamine, captopril Infection – HBV, syphilis, leprosy, filiariasis
Risk of chronic renal failure Treatment aimed at cause, can use
immunosupression Untreated 40% remission
CausesHistological classification
Nephrotic Syndrome
Minimal Change Disease
Focal segmental Glomerulosclero
sis
Membranous Glomerulonephriti
s
CausesHistological classification
Nephrotic Syndrome
Minimal Change Disease
Focal segmental Glomerulosclero
sis
Membranous Glomerulonephriti
s
Focal segmental Glomerulosclerosis
Can occur at any age Primary or secondary
Reflux IgA nephropathy Alport’s syndrome Vasculitis Sickle cell disease Heroin use HIV
50% have impaired renal function Responds to corticosteroids in 30% 30 – 50% progress to ESRF Risk of recurrence post transplant in 20 – 30%
Focal segmental Glomerulosclerosis
(A) An early lesion with segmental capillary collapse and epithelial hyperplasia (arrows).
(B and C) Glomeruli show more extensive abnormalities with on top of collapsed capillaries with epithelial hyperplasia more advanced lesions (arrowheads) with sclerosis, adhesions, epithelial hyperplasia, and mild endocapillary hypercellularity with endocapillary foam cells.
(D) A hypocellular globally sclerotic glomerulus covered with a single layer of epithelial cells, which do not appear, activated.
Investigations Urine dip
Protien ++ Granular and hyaline casts (occasional
erythrocytes) 24 urinary collection of urine , PCR
Serum albumin Serum lipid profile Serology (ASO, Antibodies, RF, ANCA, Anti
GBM) Renal ultrasound Renal biopsy
Prognosis and complications Depends on underlying disease Can be affected by degree of proteinuria,
HLA type and creatinine Complications
Hypercholesterolaemia Infection (receive oneumococcal vac) Thrombosis Renal Failure Malnutrition
Treatment
Treat underlying cause Keep blood pressure down
Corticosteroids Cyclophosphamide or ciclosporin
Minimal change Yes If frequent relapse
Membranous Based on poor prognostic factors
If renal function deteriorates
Focal segmental Yes –remission in 30%
If steroid resistant
Quiz – Who are these two?
Quiz
Linus Pauling Bright's disease 2 x Nobel prize Demonstrated that
the hemoglobin molecule changes structure when it gains or loses an oxygen
DNA triple helix?
Quiz Alonzo Harding Mourning
Played for Miami Heat
Had Focal segmental Glomerulosclerosis
Had a kidney transplant
and later won his first NBA Championship with the Heat
Thanks
Any Questions
References OHCM The renal system - Michael field, Carol Pollock,
David Harris Schieppati A, MosconiL, Perma A et al. Prognosis
of untreated patients with idiopathic membranous nephropathy. New England Journal of Medicine 1993;329: 85 -89
Tune BM, Mendoza SA. Treatment of the idiopathic nephrotic syndrome: regimens and outcomes in children and adults, Journal of the American Society of Nephrology 1997; 8: 824 - 832