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Nephrotic Syndrome Dr Ashutosh Ojha Reader, Internal Medicine 17 Feb2016 Dr A Ojha
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Dr A Ojha
Nephrotic SyndromeDr Ashutosh Ojha
Reader, Internal Medicine
17 Feb2016
Dr A Ojha
Clinical complex characterized by a number of renal and extra renal features.The most prominent being.
Proteinuria of > 3.5 g/d Hypoalbuminaemia Oedema Hyperlipidaemia Hypercoagulability.
NEPHROTIC SYNDROME
17 Feb2016
Dr A Ojha
Key component is proteinuria◦ Results from altered permeability of glomerular
filtration barrier(GBM,podocytes& their slit diaphragm) for proteins
◦ All other metabolic complications are secondary to urine protein loss.
Nephrotic Syn…….Intro
17 Feb2016
Dr A Ojha
Proteinuria Loss of lipid metabolism Sr Albumin regulating
protein Oncotic pressure Oedema
Hepatic lipoprotein synthesis
Hyperlipdaemia
Nephrotic Syn…….Intro
17 Feb2016
Dr A Ojha
Altered level of Urinary loss of protein C and S Antithrombin III
Hyperfibrinogenaemia Impaired fibrinolysis Platelet aggregability Hypercoagulability
Peripheral arterial and venous thrombosis
Renal vein thrombosis Pulmonary embolism
Manifestations….
17 Feb2016
Dr A Ojha
Other metabolic complications.◦ Protein malnutrition◦ Iron resistant microcytic hypochromic anaemia◦ Hypocalcaemia◦ Vit D deficiency◦ Secondary hypoparathyroidism◦ thyroxin levels◦ IgG
Other metabolic complications
17 Feb2016
Dr A Ojha
Damage to GBM Number & size of pores Passage of more and larger
proteins
in fixed negatively charged components
( Repel negatively charged protein molecules )
Basic mechanism
17 Feb2016
Dr A Ojha
Spectrum of Glomerular Disease
17 Feb2016
Dr A Ojha
6 entities account for >90% in adults◦ Minimal change disease(MCD)◦ Focal & segmental glomerulosclerosis(FSGS)◦ Membranous glomerulopathy.◦ Membrnoproliferative GN (MPGN).◦ Diabetic nephropathy.◦ Amyloidosis
Causes
17 Feb2016
Dr A Ojha
Systemic vasculitis eg-SLE Drugs
◦ Penicillamine◦ Captopril◦ Gold◦ Cadmium
Allergic reactions◦ Pollens◦ Bee sting
Other causes
17 Feb2016
Dr A Ojha
History.◦ Frothy urine –heavy proteinuria◦ Oedema-Periorbital,arms.ascites,genital oedema.◦ BP in some.◦ Features of underlying disease viz SLE,DM ◦ Rule out
Primary cardiac failure Chronic liver disease
General clinical features
17 Feb2016
Dr A Ojha
Diagnosis established by◦ 24 hr urinary protein >3.5g/day◦ Sr Albumin <3g/dl
Investigations
17 Feb2016
Dr A Ojha
Cholesterol, LDL, triglycerides Urea, Creatinine ---- progressive disease Urine microscopy—RBC,RBC casts Throat swab Streptococcal infection ASO titer C3 level-immune complex mediated ANA HBsAg&HCAntibodies Cryoglbulinaemia
Investigations
17 Feb2016
Dr A Ojha
Sr electrophoresis - MM Blood glucose.—DM Selective proteinuria—MCD,diabetic
nephropathy,amyloidosis. Non selective proteinuria—Diffuse
proliferative GN
Nephrotic Syn…….Inv
17 Feb2016
Dr A Ojha
Gradual onset of acute renal failure No obvious cause of acute renal failure Heavy proteinuria Significant haematuria Clinical evidence or history of systemic
disease Prolonged oliguria
Renal biopsy
17 Feb2016
Dr A Ojha
Not required in◦ Young children with highly selective proteinuria,no
HTN,no red cells or red cell casts in urine◦ Long standing insulin dependent diabetes mellitus◦ On drugs like penicillamine.
Renal biopsy
17 Feb2016
Dr A Ojha
Specific treatment of underlying cause. Drugs- immunosuppressive therapy. General measures to control proteinuria. General measures to control complications.
Management
17 Feb2016
Dr A Ojha
80% cases < 16yrs, 20% cases in adults. Peak incidence 6-8yrs Idiopathic. Occurrence after URTI, immunization. incidence in HLA B12. May be associated with interstitial nephritis,
lymphomas.
Minimal change disease
17 Feb2016
Dr A Ojha
High mol wt proteinuria. Benign urinary sediment. Microscopic haematuria in 20-30% cases. HTN & RF -----rare
presentation
17 Feb2016
Dr A Ojha
Glomerular size & structure – normal in light microscopy.
Immunoflorescense studies –neg for Ig & C3 Electron microscopy Diffuse effacement of foot processes of
visceral epithelial cells.
MCD….pathology
17 Feb2016
Dr A Ojha
MCD-Biopsy-------Normal
17 Feb2016
Dr A Ojha
Highly steroid responsive . Excellent prognosis. Spontaneous remission in 30-40% children. Remission with 8 wks of high dose
glucocorticoids in 90% of children, 50% of adults.
MCD…..treatment
17 Feb2016
Dr A Ojha
Dose Children: 60 mg/mt2/day x 4 wks
40 mg/mt2/day x 4 wks
Adults: 1.15mg/kg/day x 4 wks
1mg/kg/day x 4 wks 50% relapse fallowing withdrawal.
MCD…..treatment
17 Feb2016
Dr A Ojha
Relapse during / shortly after withdrawal Relapse - >3 / yr Cyclophosphamide- 2-3 mg/ kg /day 8 –12
wks or Chlormabucil- 0.1-0.2 mg/ kg /day 8 –12 wks
Side effects-infertility, cystitis, alopecia, infections, sec malignancies.
Cyclosporin can be tried in those resistant to above .
MCD…..Relapse & Rx
17 Feb2016
Dr A Ojha
Sclerosis with hyalinosis involving portions of < 50% glomeruli in a tissue section.
1/3 cases of nephrotic syndrome in males. Proteinuria with HTN and mild renal
insufficiency.
FSGS
17 Feb2016
Dr A Ojha
FSGS……Histology
17 Feb2016
Dr A Ojha
Spontaneous remission rare. Prognosis poor. Drug therapy unsatisfactory. Renal transplant.
Treatment
17 Feb2016
Dr A Ojha
30-40% of NS in adults. Rare in children. Peak incidence 30-50 yrs. 1/3 rd assocition with systemic diseases like
SLE, infections, malignancy, HBV, drugs.
Membranous glomerulonephritis
17 Feb2016
Dr A Ojha
Membranous Nephropathy
17 Feb2016
Dr A Ojha
Light microscopy- diffuse thickening of GBM with e/o cellular infiltration.
Electron microscopy- immune deposits of IgG & C3
pathology
17 Feb2016
Dr A Ojha
Proteinuria- non selective Microscopic hematuria in 50%. RBC casts, leukocytes, macroscopic
hematuria are rare. HTN 10-30%. 40% remit spontaneously, 10-20% slowly
progressive ESRD, others remit & relapse.
presentation
17 Feb2016
Dr A Ojha
Steroids- unsatisfactory. Cytotoxic drugs- some benefit.
treatment
17 Feb2016
Dr A Ojha
Features-Thickening of GBM- proliferative changes.-diffuse increase in mesangial
cells and matrix.-immune deposits- C3, IgG, IgM,
IgA.
mpgn
17 Feb2016
Dr A Ojha
In association with-IE-HIV-HBV, HCV-Cryoglobulinaemia-Lymphoma
presentation
17 Feb2016
Dr A Ojha
Heavy proteinuria Active urinary sediment Normal to mildly impaired renal function.
Course- benign to ESRD. No effective treatment.
presentation
17 Feb2016
Dr A Ojha
ACE inhibitors- act by ↓ intraglomerular pressure and prevent hemodynamically mediated focal segmental glomerulosclerosis.
Specially in DM, FSGS.NSAIDS- act by altering glomerular
hemodynamics& permeability.
Management of proteinuria
17 Feb2016
Dr A Ojha
Edema- salt restriction 1-2 gms/ day Loop diuretics Hyperlipidaemia- statins Thromboembolism- anticoagulation. Malnutrition- ? High protein diet. Vitamins D supplements
Management of complications
17 Feb2016
Dr A Ojha
Thank You
17 Feb2016
