Nephrotic syndrome

Nephrotic syndrome

      Nephrotic syndrome is characterised by generalised edema ,massive proteinuria ,hypoalbuminemia ,hyperlipidemia and lipiduria

Causes

Primary-

                   Minimal change nephropathy(Common for children)  

                   Membranous glomerulonephritis

                   Mesangiocapillary glomerulonephritis

                   Proliferative  glomerulonephritis

                   Focal segmental glomerulosclerosis

                   IgA nephropathy

Secondary-

                   Diabetic

                   Collagen disease-SLE ,RA

                   Amyloidosis

                   Drugs-Penicillamine ,gold

                   Bronchial carcinoma

Why edema occurs-Na/water retention ,change in molecular barrier

Why proteinuria-Increased  permeability of glomerular capillary membrane due to-

                    Loss of fixed -ve charged protein

                   Damage to glomerular basement membrane

Why hyperlipidemia-Hypoalbuminemia>Increased lipoprotein synthesis by liver ,reduced chylomicron/VLDL clearance

Why lipid abnormalities-Hypercholesterolemia ,raised LDL,VLDL ,IDL

BP-Normal.If high ,it is due to other causes like SLE ,polyarteritis nodosa ,diabetic nephropathy ,terminal stage of nephrotic syndrome

Why renal vein thrombosis/DVT/pul embolism-Loss of  anti thrombin 3 ,plasminogen in urine ,increased clotting factor synthesis

Why atherosclerosis-Hypercholesterolemia

Why infection-Loss of IgG

Why osteomalacia-Loss of  vit-D binding protein

Why Fe deficiency anemia-Loss of transferrin

          

Clinical features

• Whole body swelling(First appear on face and around eye>then arms>abdomen>leg>generalised)
• Scanty micturition
• Weakness
• Los of apetite

Differential diagnosis

                 Acute glomerulonephritis

                 Congestive cardiac failure

                 Cirrhosis

                 Hypoproteinemia due to malnutrition/malabsorption

Important history to be taken

                 Sore throat/skin infection(To exclude acute glomerulonephritis)

                 Breathlessness/cough/chest pain(To exclude congestive cardiac failure)

                 Bowel disorder(To exclude malabsorption)

                 Cold intolerance(To exclude hypothyroidism)

                 Jaundice(To exclude cirrhosis)

                 DM(To exclude diabetic nephropathy)

                 Drug history

Investigation

                 Urine RME-Massive proteinuria ,no RBC/RBC cast ,sugar(Diabetic nephropathy)

                 24 hrs urine total protein(>3.5g/24hrs)

                 Lipid profile

                 Blood sugar ,creatinine ,urea ,RFT(eGFR>CKD) ,LFT ,ECG ,PBF/iron profile

                 ANA ,Anti dsDNA(SLE)

                 USG whole abdomen-(Normally isoechoic)

                                   Glomerulonephritis-Increased cortical thickness

                                   Diabetic nephropathy-Increased cortical+medullary thickness

                                   CKD-Shrinked kidney(Fibrosis) ,decreased cortical and medullary thickness

                                             Increased cortical echogenicity

                  Renal biopsy(Types of GN) 

                 

Treatment

• Fluid restriction
• Salt restriction
• High protein diet(2g/kg/day)-Restricted if impaired renal function
• ACEi(All types GN as antiproteinuric)
• For Minimal change disease-Prednisolone 60mg/m.sq.(upto 80mg/day) for 4-6 weeks followed by 40mg/m.sq every other day for again 4-6 weeks.If relapse add cyclophosphamide

• For Membrabous glomerulonephritis-Inj.methylprednisolone 500mg for 3 days followed by oral prednisolone 0.5mg/kg/day for 27 days in 1st ,3rd and 5th months and cyclophosphamide 2mg/kg/day for 30 days in 2nd ,4th and 6th month

Prognosis

• Minimal change-Good. No chance of CKD
• Membranous glomerulonephritis-1/3rd remission ,1/3rd remain NS ,1/3rd progressive renal impairement

Complication

• Hypercoagulability(DVT/Renal/pulmonary embolism)
• Infection(Spontaneous bacterial peritonitis)
• Atherosclerosis
• Oliguric renal failure
• Fe deficiency anemia
• Osteomalacia
• Bilateral pleural/pericardial effusion

Membranous glomerulonephritis-Hematuria ,loin pain  due to renal thrombosis

If so ,give heparin and warfarin

Membranous glomerulonephritis-Male>Female ,idiopathic mostly ,biopsy shows thickening of glomerular basement membrane ,matrix deposition ,IgG deposit

Minimal change disease-Associated with atopy.No abnormality ,no deposit ,on electron microscope fusion of podocytes