Definition

  • What is nephrotic syndrome? A, hl
  • What is massive proteinuria? Hl
    • What is nephrotic range proteinuria? Hl
    • What is normal daily excretion of protein in an adult? Hl
    • How do you classify proteinuria? Hl
    • What is selective and non-selective proteinuria? Hl
    • What is microalbuminuria? hl
  • What is minimal change lesion? Hl
  • What is relapse in nephrotic syndrome? Hl
  • What are the clinical types of nephrotic syndrome? Hl
    • What is frequent relapse in nephrotic syndrome? Hl
    • What are infrequent relapses in nephrotic syndrome? Hl
    • What is steroid dependent in nephrotic syndrome? Hl
    • What do you mean by steroid resistant in nephrotic syndrome? Hl
  • Which glomerulopathies present as nephrotic syndrome? H36
  • Which glomerulopathies present as nephritonephrotic syndrome? H37

Epidemiology

  • Q

Etiology and Pathophysiology

  • What are the mechanisms of nephrotic syndrome? H41
  • What are the causes? A
  • What are the common histological types of NS? H63
  • What are the common causes of NS in children? Hl
  • What is the most common cause of nephrotic syndrome in children? Hl
  • What are the characteristics of minimal lesion disease? hl
  • What is the most common cause of nephrotic syndrome in adults? Hl
  • What are the characteristics of membranous nephropathy? hl
  • What are the lipid abnormalities in NS? What are the mechanisms? A
  • What is the mechanism of proteinuria? A
  • Why edema in NS? A
  • What is the blood pressure in NS? A
  • What are the causes of hypertension in NS? Hl
  • Which primary glomerular disease is commonly associated with hypertension? hl
  • What are the mechanisms of renal vein thrombosis in nephrotic syndrome? a

Clinical manifestations

  • What is the presentation of nephrotic syndrome? H67
  • What are the features of nephrotic syndrome? H34

Examinations

  • Q

Investigations

  • What investigations should be done in nephritic syndrome? A, hl
  • What are the findings in blood investigations in case of nephrotic syndrome? Hl
  • What are the indications of renal biopsy in nephrotic syndrome? Hl
  • What are the indications of renal biopsy? hl

Diagnosis

  • Why do you consider this diagnosis? Hl
  • What are your differential diagnoses? A, h72
  • Why not acute glomerulonephritis? A, hl
  • Why not congestive cardiac failure? A
  • Why not cirrhosis of liver? A
  • What history you have taken in NS? a

Treatment

  • How to treat nephrotic syndrome? A, hl
  • How can you treat initial attack of nephrotic syndrome? hl
  • How can you treat frequent relapse of nephrotic syndrome? hl
  • How can you follow up this patient? hl
  • What is the prognosis in NS? A, hl
  • What is the prognosis of membranous nephropathy? Hl
  • What is the prognosis of FSGS? Hl
  • Counselings in case of nephrotic syndrome. Hl
  • What is urinary remission in nephrotic syndrome? Hl
  • What is complete remission in nephrotic syndrome? hl

Complications

  • What are the complications of nephrotic syndrome? A, hl
  • How can you assess that CKD has developed in a patient of NS? hl

Discussion a

  • Membranous glomerulopathy
  • Minimal change nephropathy
  • Focal segmental glomerulosclerosis
Solve

Etiology and Pathophysiology

What are the mechanisms of nephrotic syndrome?

Mechanism of nephrotic syndrome are:
– Injury to podocytes
– Changed architecture—Scarring
– Deposition of matrix or other elements.

What are the causes of nephrotic syndrome?

Causes are:

  1. Primary renal disease—all types of glomerulonephritis (80%):
    1. Minimal change glomerular disease (the most common in children)
    2. Membranous GN (the most common in adult)
    3. Mesangiocapillary and proliferative gomerulonephritis
    4. Focal and segmental glomerulosclerosis
    5. IgA nephropathy.
  2. Secondary to other disease:
    1. Diabetic nephropathy
    2. Collagen disease, mainly SLE, also rheumatoid arthritis (by amyloidosis)
    3. Amyloidosis
    4. Drugs—penicillamine (common), captopril, gold, mercury, NSAIDs, stibogluconate.
    5. Neoplastic—carcinoma (bronchial carcinoma, ca. breast, ca. colon), lymphoma, leukemia.
    6. Infection—malaria (quartan malaria), bacterial endocarditis, HBV, HCV, HIV, secondary syphilis, leprosy, toxoplasma.
    7. Allergies—bee stings, snake bite, anti-snake venom, pollens.
*Long Cases in Clinical Medicine, ABM Abdullah;  Short and Long Cases in Clinical Medicine, HN Sarker; Step on to Paediatrics, Md Abid Hossain Mollah
What are the common histological types of NS?

– Minimal change glomerular disease
– Membranous GN
– Mesangiocapillary and proliferative gomerulonephritis

What are the common causes of NS in children?

Causes are:

  1. Minimal change glomerular disease (70%)
  2. Mesangiocapillary and proliferative gomerulonephritis (15%)
  3. Focal and segmental glomerulosclerosis (10%)
What is the most common cause of nephrotic syndrome in children?
Minimal change glomerular disease is the most common in children (70%)
What are the characteristics of minimal lesion disease?
  • Common in children, particularly male, but may occur in all ages, associated with atopy
  • On light microscopy, there is no abnormality but on electron microscopy, there is fusion of podocyte foot process (flattening of the foot process)
  • Selective proteinuria
  • No immune deposit
  • Good response to steroid and cytotoxic drugs.
  • Progress is excellent, progression to renal failure is rare
*Long Cases in Clinical Medicine, ABM Abdullah; Short and Long Cases in Clinical Medicine, HN Sarker
What is the most common cause of nephrotic syndrome in adults?
Membranous nephropathy is the most common in adult.
What are the characteristics of membranous nephropathy?
  • It is a common cause of NS in adult (35%), predominantly in males
  • It is mostly idiopathic
  • May be secondary to—SLE, bronchial carcinoma, drugs (penicillamine), heavy metals like mercury, HBV, HCV
  • There is granular subepithelial IgG deposit
  • Non-selective proteinuria
  • Renal biopsy shows thickening of glomerular basement membrane, increased matrix deposition and glomerulosclerosis
  • Response to steroid and other cytotoxic drugs is less.
  • Prognosis is bad
  • Renal vein thrombosis is a common complication
  • May progress to CKD (10-15%)
*Long Cases in Clinical Medicine, ABM Abdullah; Short and Long Cases in Clinical Medicine, HN Sarker
What are the lipid abnormalities in NS? What are the mechanisms?

Lipid abnormalities are:

  • Hypercholesterolemia and hypertriglyceridemia
  • High LDL, VLDL and IDL
  • There is no change or low HDL.

The mechanisms are:

  1. Increased synthesis of lipoproteins by the liver, secondary to hypoalbuminemia
  2. Reduced clearance of triglyceride bearing lipoprotein (chylomicron and VLDL) in direct response to albuminuria.
    As a result, there is high rate of atherosclerosis.
*Long Cases in Clinical Medicine, ABM Abdullah
What is the mechanism of proteinuria?

There is increased permeability of the glomerular capillary wall due to –

  1. Reduction of fixed negatively charged protein molecules in glomerular capillary wall which repel negatively charged protein molecules, allows proteins to pass through the pores
  2. Damage to the glomerular basement membrane that leads to increase in the size and number of pores, allowing passage of larger molecules.
*Long Cases in Clinical Medicine, ABM Abdullah
Why edema in NS?

Previously it was thought that reduction of albumin causes low plasma oncotic pressure, which causes to edema. But recent view is oncotic pressure is not changed in NS, edema is due to sodium retention in the extracellular compartment. Also, there is change in molecular barrier, which causes edema.

*Long Cases in Clinical Medicine, ABM Abdullah
What is the blood pressure in NS?

Usually blood pressure is normal, even low. If there is hypertension, usually it is secondary to underlying disease like SLE with renal involvement, polyarteritis nodosa, diabetic nephropathy or terminal stage of nephrotic syndrome.

*Long Cases in Clinical Medicine, ABM Abdullah
What are the causes of hypertension in NS?

Usually hypertension is not a feature of NS. If present, one should consider:

  • Onset of CRF
  • NS in SLE
  • NS in polyarteritis nodosa,
  • Kimmelstiel-Wilson (K-W) syndrome (NS in DM)
*Short and Long Cases in Clinical Medicine, HN Sarker
Which primary glomerular disease is commonly associated with hypertension?
Focal segmental glomerulosclerosis (FSGS).
What are the mechanisms of renal vein thrombosis in nephrotic syndrome?

Mechanisms are as follows:

  1. In nephrotic syndrome, there is hypercoagulable state due to:
    • Loss of inhibitors of coagulation in urine, such as antithrombin III, protein C and S, and also loss of fibrinolytic factor (plasminogen).
    • Increase synthesis of clotting factors—factor V, VIII and fibrinogen.
    • Other factors—thrombocytosis and over diuresis resulting in dehydration, reduced renal blood flow and increased viscosity, prolonged bed ridden.
  2. Also, there is hyperlipidemia, commonly high LDL, VLDL, cholesterol and triglyceride. So, there is more atherosclerosis.
    These predispose to increased venous thrombosis that occurs specially in renal vein.
*Long Cases in Clinical Medicine, ABM Abdullah.
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